We report a case of Wilms tumour associated with unilateral partial aniridia. Although Wilms tumour is known to be associated with bilateral aniridia, there is only one previously reported case associated with dysplasia of the iris.
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The Wilms' Tumor Suppressor WT1 in Cardiomyocytes: Implications for Cardiac Homeostasis and Repair.
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December 2024
Université Côte d'Azur, CNRS, INSERM, iBV, 06107 Nice, France.
The Wilms' tumor suppressor WT1 is essential for the development of the heart, among other organs such as the kidneys and gonads. The Wt1 gene encodes a zinc finger transcription factor that regulates proliferation, cellular differentiation processes, and apoptosis. WT1 is also involved in cardiac homeostasis and repair.
View Article and Find Full Text PDFEx Vivo Fluorescence Confocal Microscopy Meets Innovation and Revolutionary Technology, for "Real-Time" Histological Evaluation, in Pediatric Surgical Oncology.
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November 2024
Pediatric Surgery Unit, Salesi Children's Hospital, Polytechnic University of Marche, Via Filippo Corridoni, 16, 60123 Ancona, Italy.
Background And Aim: Ex vivo fluorescence confocal microscopy (FCM) systems are innovative optical imaging tools that create virtual high-resolution histological images without any standard tissue processing, either freezing or fixing in formalin and embedding in paraffin. These systems have opened an era that would revolutionize pathological examination by providing rapid, real-time assessments across various pathology subspecialties, potentially replacing conventional methods that are tissue- and time-consuming. This study aimed to present the first utilization of FCM in pediatric surgical oncology, focusing on assessing the benefits, particularly in facilitating rapid and accurate diagnosis.
View Article and Find Full Text PDFAPOL1 Modulates Renin-Angiotensin System.
Biomolecules
December 2024
Department of Medicine and Feinstein Institute for Medical Research, Zucker School of Medicine, Hempstead, NY 11549, USA.
Patients carrying APOL1 risk alleles (G1 and G2) have a higher risk of developing Focal Segmental Glomerulosclerosis (FSGS); we hypothesized that escalated levels of miR193a contribute to kidney injury by activating renin-angiotensin system (RAS) in the APOL1 milieus. Differentiated podocytes (DPDs) stably expressing vector (V/DPD), G0 (G0/DPDs), G1 (G1/DPDs), and G2 (G2/DPDs) were evaluated for renin, Vitamin D receptor (VDR), and podocyte molecular markers (PDMMs, including WT1, Podocalyxin, Nephrin, and Cluster of Differentiation [CD]2 associated protein [AP]). G0/DPDs displayed attenuated renin but an enhanced expression of VDR and Wilms Tumor [WT]1, including other PDMMs; in contrast, G1/DPDs and G2/DPDs exhibited enhanced expression of renin but decreased expression of VDR and WT1, as well as other PDMMs (at both the protein and mRNA levels).
View Article and Find Full Text PDFNephron-sparing Surgery for Pediatric Renal Tumors After Centralization of Pediatric Oncology Care in the Netherlands: Improved Outcomes With 3D Modeling.
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December 2024
Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands.
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Acute myeloid leukemia (AML) that is relapsed and/or refractory post-allogeneic hematopoietic cell transplantation (HCT) is usually fatal. In a prior study, we demonstrated that AML relapse in high-risk patients was prevented by post-HCT immunotherapy with Epstein-Barr virus (EBV)-specific donor CD8 T cells engineered to express a high-affinity Wilms Tumor Antigen 1 (WT1)-specific T-cell receptor (TTCR- C4). However, in the present study, infusion of EBV- or Cytomegalovirus (CMV)-specific T did not clearly improve outcomes in fifteen patients with active disease post-HCT.
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