Recently added

DSpace/Manakin Repository

UMC Repository: Recent submissions

  • PPARgamma in Metabolism, Immunity, and Cancer: Unified and Diverse Mechanisms of Action 
    Hernandez-Quiles, Miguel; Broekema, Marjoleine F.; Kalkhoven, Eric (Frontiers Media S. A., 2021-02-26)
    The proliferator-activated receptor γ (PPARγ), a member of the nuclear receptor superfamily, is one of the most extensively studied ligand-inducible transcription factors. Since its identification in the early 1990s, PPARγ ...
  • Comprehensive profiling of mammalian tribbles interactomes implicates TRIB3 in gene repression 
    TRAIN Consortium (Multidisciplinary Digital Publishing Institute (MDPI), 2021-12-01)
    The three human Tribbles (TRIB) pseudokinases have been implicated in a plethora of signaling and metabolic processes linked to cancer initiation and progression and can potentially be used as biomarkers of disease and ...
  • Significant variation in histopathological assessment of endoscopic resections for Barrett's neoplasia suggests need for consensus reporting: propositions for improvement 
    van der Wel, M. J.; Klaver, E.; Pouw, R. E.; Brosens, L. A.A.; Biermann, K.; Doukas, M.; Huysentruyt, C.; Karrenbeld, A.; Ten Kate, F. J.W.; Kats-Ugurlu, G.; van der Laan, J.; van Lijnschoten, I.; Moll, F. C.P.; Offerhaus, G. J.A.; Ooms, A. H.A.G.; Seldenrijk, C. A.; Visser, M.; Tijssen, J. G.; Meijer, S. L.; Bergman, J. J.G.H.M. (Wiley-Blackwell, 2021-12-24)
    Endoscopic resection (ER) is an important diagnostic step in management of patients with early Barrett's esophagus (BE) neoplasia. Based on ER specimens, an accurate histological diagnosis can be made, which guides further ...
  • Superficial basal cell carcinoma, think deeper: Step sectioning of skin biopsy specimens yields 14% more aggressive subtypes 
    El Sharouni, Mary-Ann; van Diest, Paul J; Blokx, Willeke A M (Public Library of Science, 2022-01)
    INTRODUCTION: Because of different therapeutic regimens for superficial and non-superficial basal cell carcinomas (BCCs), accurate histopathological examination of a punch biopsy to determine its subtype is essential. The ...
  • Landscape of driver gene events, biomarkers, and druggable targets identified by whole-genome sequencing of glioblastomas 
    van de Geer, Wesley S; Hoogstrate, Youri; Draaisma, Kaspar; Robe, Pierre A; Bins, Sander; Mathijssen, Ron H J; French, Pim; van de Werken, Harmen J G; de Vos, Filip Y F (Oxford University Press, 2022-01-21)
    Background: The survival of glioblastoma patients is poor. Median survival after diagnosis is 15 months, despite treatment involving surgical resection, radiotherapy, and/or temozolomide chemotherapy. Identification of ...
  • Serum biomarkers confirming stable remission in inflammatory bowel disease 
    Kessel, Christoph; Lavric, Miha; Weinhage, Toni; Brueckner, Markus; de Roock, Sytze; Däbritz, Jan; Weber, Jakob; Vastert, Sebastiaan J.; Foell, Dirk (Nature Publishing Group, 2021-03)
    Crohn's disease (CD) and ulcerative colitis (UC) have a chronic-remittent course. Optimal management of inflammatory bowel diseases (IBD) relies on early intervention, treat-to-target strategies and a tight disease control. ...
  • Thrombotic Microangiopathy Associated with Macrophage Activation Syndrome: A Multinational Study of 23 Patients 
    the MAS/sJIA Working Group of the Pediatric Rheumatology European Society (PReS) (Mosby Inc., 2021-08)
    Objective: To describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA). Study design: International ...
  • Definition and validation of serum biomarkers for optimal differentiation of hyperferritinaemic cytokine storm conditions in children: a retrospective cohort study 
    Kessel, Christoph; Fall, Ndate; Grom, Alexei; de Jager, Wilco; Vastert, Sebastiaan; Strippoli, Raffaele; Bracaglia, Claudia; Sundberg, Erik; Horne, Anna Carin; Ehl, Stephan; Ammann, Sandra; Wouters, Carine; Lehmberg, Kai; De Benedetti, Fabrizio; Park, Carolin; Hinze, Claas; Wittkowski, Helmut; Kessel, Katharina; Beutel, Karin; Foell, Dirk; Holzinger, Dirk (Elsevier Limited, 2021-08)
    Background: Cytokine storm syndromes are life-threatening complications that can occur in children with rheumatic conditions (macrophage activation syndrome [MAS]), inherited cytotoxicity defects (ie, primary haemophagocytic ...
  • Definition and Validation of the American College of Rheumatology 2021 Juvenile Arthritis Disease Activity Score Cutoffs for Disease Activity States in Juvenile Idiopathic Arthritis 
    for the Paediatric Rheumatology International Trials Organisation (John Wiley & Sons Inc., 2021-11)
    Objective: To develop and validate new Juvenile Arthritis Disease Activity Score 10 (JADAS10) and clinical JADAS10 (cJADAS10) cutoffs to separate the states of inactive disease (ID), minimal disease activity (MiDA), moderate ...
  • Pathogenesis and Treatment of Refractory Disease Courses in Systemic Juvenile Idiopathic Arthritis: Refractory Arthritis, Recurrent Macrophage Activation Syndrome and Chronic Lung Disease 
    Erkens, Remco; Esteban, Ysabella; Towe, Christopher; Schulert, Grant; Vastert, Sebastiaan (W.B. Saunders, 2021-11)
    Systemic juvenile idiopathic arthritis is a distinct and heterogeneous disease presently classified under the umbrella of juvenile idiopathic arthritis, with some patients following a monophasic remitting course, whereas ...
  • Hematopoietic Cell Transplantation Cures Adenosine Deaminase 2 Deficiency: Report on 30 Patients 
    Hashem, Hasan; Bucciol, Giorgia; Ozen, Seza; Unal, Sule; Bozkaya, Ikbal Ok; Akarsu, Nurten; Taskinen, Mervi; Koskenvuo, Minna; Saarela, Janna; Dimitrova, Dimana; Hickstein, Dennis D.; Hsu, Amy P.; Holland, Steven M.; Krance, Robert; Sasa, Ghadir; Kumar, Ashish R.; Müller, Ingo; de Sousa, Monica Abreu; Delafontaine, Selket; Moens, Leen; Babor, Florian; Barzaghi, Federica; Cicalese, Maria Pia; Bredius, Robbert; van Montfrans, Joris; Baretta, Valentina; Cesaro, Simone; Stepensky, Polina; Benedicte, Neven; Moshous, Despina; Le Guenno, Guillaume; Boutboul, David; Dalal, Jignesh; Brooks, Joel P.; Dokmeci, Elif; Dara, Jasmeen; Lucas, Carrie L.; Hambleton, Sophie; Wilson, Keith; Jolles, Stephen; Koc, Yener; Güngör, Tayfun; Schnider, Caroline; Candotti, Fabio; Steinmann, Sandra; Schulz, Ansgar; Chambers, Chip; Hershfield, Michael; Ombrello, Amanda; Kanakry, Jennifer A. (Springer New York, 2021-10)
    Purpose: Deficiency of adenosine deaminase 2 (DADA2) is an inherited inborn error of immunity, characterized by autoinflammation (recurrent fever), vasculopathy (livedo racemosa, polyarteritis nodosa, lacunar ischemic ...
  • Abnormal Results of Newborn Screening for SCID After Azathioprine Exposure In Utero: Benefit of TPMT Genotyping in Both Mother and Child 
    Blom, Maartje; Pico-Knijnenburg, Ingrid; van Montfrans, Joris M.; Bredius, Robbert G.M.; van der Burg, Mirjam; Swen, Jesse J.; Berghuis, Dagmar (Springer New York, 2021-10-07)
  • The pulmonary metatranscriptome prior to pediatric HCT identifies post-HCT lung injury 
    Zinter, Matt S.; Lindemans, Caroline A.; Versluys, Birgitta A.; Mayday, Madeline Y.; Sunshine, Sara; Reyes, Gustavo; Sirota, Marina; Sapru, Anil; Matthay, Michael A.; Kharbanda, Sandhya; Dvorak, Christopher C.; Boelens, Jaap J.; DeRisi, Joseph L. (Elsevier, 2021-03-25)
    Lung injury after pediatric allogeneic hematopoietic cell transplantation (HCT) is a common and disastrous complication that threatens long-term survival. To develop strategies to prevent lung injury, novel tools are needed ...
  • Allogenic Hematopoietic Stem Cell Transplantation Is Feasible in Pediatric Patients with an Active or Recently Diagnosed Invasive Fungal Infection 
    Rotte, Laura G.Y.; Loeffen, Yvette G.T.; Bierings, Marc B.; Wolfs, Tom F.W.; Lindemans, Caroline A. (Elsevier, 2021-09)
    Data on the outcome of allogenic hematopoietic stem cell transplantation (HSCT) in pediatric patients with a history of invasive fungal infection (IFI) are limited. The aim of this study was to report on the feasibility ...
  • Hematopoietic dysfunction during graft-versus-host disease: A self-destructive process? 
    Müskens, Konradin F.; Lindemans, Caroline A.; Belderbos, Mirjam E. (Multidisciplinary Digital Publishing Institute (MDPI), 2021-08-10)
    Graft-versus-host disease (GvHD) is a major complication of allogeneic hematopoietic (stem) cell transplantation (HCT). Clinically, GvHD is associated with severe and long-lasting hematopoietic dysfunction, which may ...
  • Hematopoietic cell transplantation in severe combined immunodeficiency: The SCETIDE 2006-2014 European cohort 
    Lankester, Arjan C.; Neven, Benedicte; Mahlaoui, Nizar; von Asmuth, Erik G.J.; Courteille, Virginie; Alligon, Mikael; Albert, Michael H.; Serra, Isabelle Badell; Bader, Peter; Balashov, Dmitry; Beier, Rita; Bertrand, Yves; Blanche, Stephane; Bordon, Victoria; Bredius, Robbert G.; Cant, Andrew; Cavazzana, Marina; Diaz-de-Heredia, Cristina; Dogu, Figen; Ehlert, Karoline; Entz-Werle, Natacha; Fasth, Anders; Ferrua, Francesca; Ferster, Alina; Formankova, Renata; Friedrich, Wilhelm; Gonzalez-Vicent, Marta; Gozdzik, Jolanta; Güngör, Tayfun; Hoenig, Manfred; Ikinciogullari, Aydan; Kalwak, Krzysztof; Kansoy, Savas; Kupesiz, Alphan; Lanfranchi, Arnalda; Lindemans, Caroline A.; Meisel, Roland; Michel, Gerard; Miranda, Nuno A.A.; Moraleda, Jose; Moshous, Despina; Pichler, Herbert; Rao, Kanchan; Sedlacek, Petr; Slatter, Mary; Soncini, Elena; Speckmann, Carsten; Sundin, Mikael; Toren, Amos; Vettenranta, Kim (Mosby Inc., 2022-05)
    Background: Hematopoietic stem cell transplantation (HSCT) represents a curative treatment for patients with severe combined immunodeficiency (SCID), a group of monogenic immune disorders with an otherwise fatal outcome. ...
  • Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study 
    Beroukhim, Rebecca S; Ghelani, Sunil; Ashwath, Ravi; Balasubramanian, Sowmya; Biko, David M; Buddhe, Sujatha; Campbell, M Jay; Cross, Russell; Festa, Pierluigi; Griffin, Lindsay; Grotenhuis, Heynric; Hasbani, Keren; Hashemi, Sassan; Hegde, Sanjeet; Hussain, Tarique; Jain, Supriya; Kiaffas, Maria; Kutty, Shelby; Lam, Christopher Z; Liberato, Gabriela; Merlocco, Anthony; Misra, Nilanjana; Mowers, Katie L; Muniz, Juan Carlos; Nutting, Arni; Parra, David A; Patel, Jyoti K; Perez-Atayde, Antonio R; Prasad, Deepa; Rosental, Carlos F; Shah, Amee; Samyn, Margaret M; Sleeper, Lynn A; Slesnick, Timothy; Valsangiacomo, Emanuela; Geva, Tal (Elsevier, 2022-08)
    BACKGROUND: After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac ...
  • Detectable A Disintegrin and Metalloproteinase With Thrombospondin Motifs-1 in Serum Is Associated With Adverse Outcome in Pediatric Sepsis 
    Boeddha, Navin P; Driessen, Gertjan J; Hagedoorn, Nienke N; Kohlfuerst, Daniela S; Hoggart, Clive J; van Rijswijk, Angelique L; Ekinci, Ebru; Priem, Debby; Schlapbach, Luregn J; Herberg, Jethro A; de Groot, Ronald; Anderson, Suzanne T; Fink, Colin G; Carrol, Enitan D; van der Flier, Michiel; Martinón-Torres, Federico; Levin, Michael; Leebeek, Frank W; Zenz, Werner; de Maat, Moniek P M; Hazelzet, Jan A; Emonts, Marieke; Dik, Willem A (Lippincott Williams & Wilkins, 2021-11)
    IMPORTANCE: A Disintegrin and Metalloproteinase with Thrombospondin Motifs-1 is hypothesized to play a role in the pathogenesis of invasive infection, but studies in sepsis are lacking. OBJECTIVES: To study A Disintegrin ...
  • Development and validation of a prediction model for invasive bacterial infections in febrile children at European Emergency Departments: MOFICHE, a prospective observational study 
    Hagedoorn, Nienke N.; Borensztajn, Dorine; Nijman, Ruud Gerard; Nieboer, Daan; Herberg, Jethro Adam; Balode, Anda; Von Both, Ulrich; Carrol, Enitan; Eleftheriou, Irini; Emonts, Marieke; Van Der Flier, Michiel; De Groot, Ronald; Kohlmaier, Benno; Lim, Emma; MacOnochie, Ian; Martinón-Torres, Federico; Pokorn, Marko; Strle, Franc; Tsolia, Maria; Zavadska, Dace; Zenz, Werner; Levin, Michael; Vermont, Clementien; Moll, Henriette A. (BMJ Publishing Group, 2021-07-01)
    Objectives To develop and cross-validate a multivariable clinical prediction model to identify invasive bacterial infections (IBI) and to identify patient groups who might benefit from new biomarkers. Design Prospective ...
  • Respiratory Tract Infection Management and Antibiotic Prescription in Children: A Unique Study Comparing Three Levels of Healthcare in the Netherlands 
    Van Aerde, Koen J.; De Haan, Liza; Van Leur, Mattijn; Gerrits, Gerardus P.; Schers, Henk; Moll, Henriette A.; Hagedoorn, Nienke N.; Herberg, Jethro A.; Levin, Michael; Rivero-Calle, Irene; De Jonge, Marien I.; De Groot, Ronald; Van Der Flier, Michiel (Lippincott Williams & Wilkins, 2021-03)
    Background: Respiratory tract infections (RTIs) are common in children with febrile illness visiting the general practitioner (GP) or emergency department. We studied the management of children with fever and RTI at 3 ...