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Wilm tumor 1

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https://www.readbyqxmd.com/read/28627003/sonographic-screening-for-wilms-tumor-in-children-with-cloves-syndrome
#1
Caitlin M Peterman, R Dawn Fevurly, Ahmad I Alomari, Cameron C Trenor, Denise M Adams, Sophie Vadeboncoeur, Marilyn G Liang, Arin K Greene, John B Mulliken, Steven J Fishman
BACKGROUND: CLOVES syndrome is associated with somatic mosaic PIK3CA mutations and characterized by congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies. Wilms tumor (WT) is a malignant embryonal renal neoplasm associated with hemihypertrophy and certain overgrowth disorders. After identifying WT in a child with CLOVES, we questioned whether ultrasonographic screening was necessary in these patients. METHODS: We retrospectively reviewed patients with CLOVES syndrome in our Vascular Anomalies Center at Boston Children's Hospital between 1998 and 2016 to identify those who developed WT...
June 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28598537/factors-possibly-affecting-prognosis-in-children-with-wilms-tumor-diagnosed-before-24-months-of-age-a-report-from-the-associazione-italiana-ematologia-oncologia-pediatrica-aieop-wilms-tumor-working-group
#2
Paolo D'Angelo, Andrea Di Cataldo, Monica Terenziani, Gianni Bisogno, Paola Collini, Martina Di Martino, Fraia Melchionda, Clara Mosa, Marilina Nantron, Daniela Perotti, Giuseppe Puccio, Annalisa Serra, Serena Catania, Filippo Spreafico
BACKGROUND: Children with Wilms' tumor (WT) aged under 24 months (infants) have a better prognosis than older patients. Our aim was to study the epidemiology of this age group, with focus on the modality of diagnosis, tumor size, and association with malformations/syndromes, seeking to understand if any of these factors might be related to prognosis. PATIENTS AND METHODS: Infants diagnosed with WT between 2003 and February 2010 were evaluated. A query form was used to collect data on the modality of WT diagnosis (symptomatic or incidental), tumor volume, maximum diameter, site, and stage...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28583549/reduction-of-slit-diaphragm-associated-molecules-by-sirolimus-is-it-enough-to-induce-proteinuria
#3
B S Kim, J G Lee, Y Cho, S H Song, K H Huh, M S Kim, Y S Kim
Sirolimus (SRL), a mammalian target of rapamycin inhibitor, is widely used in transplantation, but the mechanisms whereby it induces adverse effects, such as proteinuria and edema, remain unclear. To determine whether isolated SRL induces proteinuria or not, the authors intraperitoneally injected C57BL/6 mice with different doses of SRL (0 mg/[kg·d], 3 mg/[kg·d], 10 mg/[kg·d], or 30 mg/[kg·d]) for 24 days. Urinary albumin excretion was then quantified using a double-sandwich enzyme-linked immunosorbent assay, and serum creatinine levels were measured using a single dry-film chemistry auto-analyzer...
June 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28582410/a-phase-i-study-of-single-agent-perifosine-for-recurrent-or-refractory-pediatric-cns-and-solid-tumors
#4
Oren J Becher, Nathan E Millard, Shakeel Modak, Brian H Kushner, Sofia Haque, Ivan Spasojevic, Tanya M Trippett, Stephen W Gilheeney, Yasmin Khakoo, David C Lyden, Kevin C De Braganca, Jill M Kolesar, Jason T Huse, Kim Kramer, Nai-Kong V Cheung, Ira J Dunkel
The PI3K/Akt/mTOR signaling pathway is aberrantly activated in various pediatric tumors. We conducted a phase I study of the Akt inhibitor perifosine in patients with recurrent/refractory pediatric CNS and solid tumors. This was a standard 3+3 open-label dose-escalation study to assess pharmacokinetics, describe toxicities, and identify the MTD for single-agent perifosine. Five dose levels were investigated, ranging from 25 to 125 mg/m2/day for 28 days per cycle. Twenty-three patients (median age 10 years, range 4-18 years) with CNS tumors (DIPG [n = 3], high-grade glioma [n = 5], medulloblastoma [n = 2], ependymoma [n = 3]), neuroblastoma (n = 8), Wilms tumor (n = 1), and Ewing sarcoma (n = 1) were treated...
2017: PloS One
https://www.readbyqxmd.com/read/28573960/sensitive-detection-of-rare-antigen-specific-t-cells-directed-against-wilms-tumor-1-by-fluorospot-assay
#5
Tina Danielzik, Michael Koldehoff, Ulrike Buttkereit, Dietrich W Beelen, Peter A Horn, Monika Lindemann
No abstract text is available yet for this article.
June 2, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28567073/quantitative-assessment-of-wilms-tumor-1-expression-by-real-time-quantitative-polymerase-chain-reaction-in-patients-with-acute-myeloblastic-leukemia
#6
Hossein Ayatollahi, Mohammad Hadi Sadeghian, Mahmood Naderi, Amir Hossein Jafarian, Seyyede Fatemeh Shams, Neda Motamedirad, Maryam Sheikhi, Afsane Bahrami, Sepideh Shakeri
BACKGROUND: The Wilms tumor 1 (WT1) gene is originally defined as a tumor suppressor gene and a transcription factor that overexpressed in leukemic cells. It is highly expressed in more than 80% of acute myeloid leukemia (AML) patients, both in bone marrow (BM) and in peripheral blood (PB), and it is used as a powerful and independent marker of minimal residual disease (MRD); we have determined the expression levels of the WT1 by real-time quantitative polymerase chain reaction (RQ-PCR) in PB and BM in 126 newly diagnosed AML patients...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28551672/ctnnb1-mutations-in-ovarian-microcystic-stromal-tumors-identification-of-a-novel-deletion-mutation-and-the-use-of-pyrosequencing-to-identify-reported-point-mutation
#7
Kiyong Na, Eun Kyung Kim, Wonjun Jang, Hyun-Soo Kim
BACKGROUND/AIM: Microcystic stromal tumor (MCST) is a rare stromal tumor of the ovary. In this study, we describe clinicopathological characteristics and results of mutational analyses of the CTNNB1gene in two cases of ovarian MCST and we provide a thorough review of previously published cases alongside our current cases and clarify the clinicopathological characteristics of ovarian MCST. PATIENTS AND METHODS: Patients' age was 33 and 31 years, respectively. One patient presented with fever and low abdominal pain, whereas a pelvic mass was incidentally detected in another patient...
June 2017: Anticancer Research
https://www.readbyqxmd.com/read/28550085/knockdown-of-sodium-calcium-exchanger-1-induces-epithelial-to-mesenchymal-transition-in-kidney-epithelial-cells
#8
Sona Lakshme Balasubramaniam, Anilkumar Gopalakrishnapillai, Nicholas J Petrelli, Sonali P Barwe
Mesenchymal to epithelial transition (MET) and epithelial to mesenchymal transition (EMT) are important processes in kidney development. Failure to undergo MET during development leads to the initiation of Wilms tumor (WT) whereas EMT contributes to the development of renal cell carcinomas (RCC). The role of calcium regulators in governing these processes is becoming evident. We demonstrated earlier that Na+/Ca2+ exchanger 1 (NCX1), a major calcium exporter in renal epithelial cells, regulates epithelial cell motility...
May 26, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28544366/enumeration-of-wt1-specific-cd8-t-cells-for-clinical-application-using-an-mhc-streptamer-based-no-wash-single-platform-flow-cytometric-assay
#9
Sarah Matko, Madeleine Teichert, Antje Tunger, Marc Schmitz, Martin Bornhauser, Torsten Tonn, Marcus Odendahl
The advent of novel strategies to generate leukemia-associated-antigen (LAA)-specific T cells for adoptive immunotherapies creates a demand for standardized good laboratory practice (GLP)-compliant enumeration assays to provide a secure clinical environment-whether it is to identify potential donors, define therapeutic doses for transplantation, or monitor clinical success. Here, we introduce a no-wash assay based on single-platform cell enumeration and Streptamer staining to determine the Wilms' tumor antigen 1 (WT1)-specific T cell immunity in clinical samples...
May 25, 2017: Cytometry. Part A: the Journal of the International Society for Analytical Cytology
https://www.readbyqxmd.com/read/28542793/the-genetic-factors-contributing-to-the-development-of-wilm-s-tumor-and-their-clinical-utility-in-its-diagnosis-and-prognosis
#10
REVIEW
Afsane Bahrami, Marjan Joodi, Mina Maftooh, Gordon A Ferns, Mehrdad Ahmadi, Seyed Mahdi Hassanian, Amir Avan
Mutations in the Wilm's tumor 1 (WT1) gene are associated with a wide spectrum of renal manifestations, ultimately leading to end-stage kidney failure. There is an inadequate understanding of the molecular functions of WT1 in renal development, and this has limited the potential for therapeutic interventions in WT1-related diseases. In this review we discuss the existing data on the genetic and epigenetic abnormalities that have been described in WTs and their potential utility as biomarkers for risk stratification, prediction and prognosis in patients withWTs...
May 20, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28534317/wt1-alternative-splicing-role-of-its-isoforms-in-neuroblastoma
#11
REVIEW
Daniela Maria Rasà, Agata Grazia D'Amico, Grazia Maugeri, Sebastiano Cavallaro, Velia D'Agata
Wilms tumor 1 (WT1), a tumor suppressor gene, was originally identified in the homonymous renal neoplasm but is also involved in other cancers. Its function is still unclear, since it acts both as a pro- and an anti-oncogene. At least 14 WT1 transcriptional variants have been described; yet most investigations have focused on a small number of isoforms. We describe their structural features and review the evidence of their involvement in cancer with emphasis on neuroblastoma. In future, full characterization of all WT1 isoforms is expected to identify new molecular tumor markers and/or therapeutic targets...
June 2017: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/28529243/over-expression-of-mir-21-and-lower-pten-levels-in-wilms-tumor-with-aggressive-behavior
#12
Mingyu Cui, Wei Liu, Lijuan Zhang, Feng Guo, Yang Liu, Fang Chen, Ting Liu, Rui Ma, Rongde Wu
Wilms' tumor (WT) is the most common pediatric kidney tumor. MiR-21 is one of the most frequently overexpressed microRNAs in solid tumors, while phosphatase and tensin homolog deleted from chromosome 10 (PTEN) is the most highly mutated tumor suppressor gene. The aim of this study was to investigate the relationship between miR-21 and PTEN in WT. The expression levels of miR-21 and the PTEN protein were determined by qRT-PCR and Western blot analyses in WT specimens, respectively. In WT tissues, the miR-21 expression levels were significantly higher and the PTEN protein levels were significantly lower, compared to the adjacent non-tumorous renal tissues...
2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28527702/crispr-cas9-induced-disruption-of-wt1a-and-wt1b-reveals-their-different-roles-in-kidney-and-gonad-development-in-nile-tilapia
#13
Dongneng Jiang, Jinlin Chen, Zheng Fan, Dejie Tan, Jiue Zhao, Hongjuan Shi, Zhilong Liu, Wenjing Tao, Minghui Li, Deshou Wang
Wilms tumor 1 (Wt1) is an essential factor for urogenital system development. Teleosts have two wt1s, named as wt1a and wt1b. In this study, the expression pattern of wt1a and wt1b and their functions on the urogenital system were analyzed by in situ hybridization and CRISPR/Cas9. wt1a was found to be expressed in the glomerulus at 3 dah (days after hatching), earlier than wt1b. wt1a and wt1b were simultaneously expressed in the somatic cells of gonads at 3 dah, while their cell locations were similar, but not identical in adult fish gonads...
May 18, 2017: Developmental Biology
https://www.readbyqxmd.com/read/28521413/chromosome-t-7-11-p15-p15-translocation-in-acute-myeloid-leukemia-coexisting-with-multilineage-dyspoiesis-and-mutations-in-nras-and-wt1-a-case-report-and-literature-review
#14
Jingke Yang, Xiaodong Lyu, Xinghu Zhu, Xiangguang Meng, Wenli Zuo, Hao Ai, Mei Deng
The chromosomal translocation t(7;11)(p15;p15) and the resulting nucleoporin 98-homeobox A9 (NUP98-HOXA9) gene fusion is rare but recurrent genetic abnormity in acute myeloid leukemia (AML). The present study describes a case of AML plus maturation (-M2) with multilineage dyspoiesis in a 30-year-old male in whom a 46,XY,t(7;11)(p15;p15) karyotype was detected through chromosome analysis. Subsequent molecular and sequencing analysis demonstrated a NUP98-HOXA9 fusion gene with a type I fusion between NUP98 exon 12 and HOXA9 exon 1b, and mutations in neuroblastoma V-Ras oncogene homolog and Wilms tumor 1...
May 2017: Oncology Letters
https://www.readbyqxmd.com/read/28498419/mir-590-regulates-wt1-during-proliferation-of-g401-cells
#15
Liyi Hong, Xu Zhao, Xuejun Shao, Hong Zhu
Nephroblastoma (Wilms' tumor) is frequently associated with mortality in children. MicroRNAs (miRNAs) are important for tumor development serving as oncogenes or tumor suppressors. In the present study, miRNA‑590 (miR‑590) was identified to be upregulated in Wilms' tumor tissues compared with the normal adjacent tissues. Additionally, the levels of miR‑590 were consistent with their clinical stage. Wilms' tumor 1 (WT1) was considered to be a tumor suppressor in certain tumor types, and it has been detected at low expression levels in various types of cancer with high cell proliferation and aggressive behavior...
July 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28493961/aldosterone-signaling-regulates-the-over-expression-of-claudin-4-and-8-at-the-distal-nephron-from-type-1-diabetic-rats
#16
Eduardo Molina-Jijón, Rafael Rodríguez-Muñoz, Ricardo González-Ramírez, Carmen Namorado-Tónix, José Pedraza-Chaverri, Jose L Reyes
Hyperglycemia in diabetes alters tight junction (TJ) proteins in the kidney. We evaluated the participation of aldosterone (ALD), and the effect of spironolactone (SPL), a mineralocorticoid receptor antagonist, on the expressions of claudin-2, -4, -5 and -8, and occludin in glomeruli, proximal and distal tubules isolated from diabetic rats. Type 1 diabetes was induced in female Wistar rats by a single tail vein injection of streptozotocin (STZ), and SPL was administrated daily by gavage, from days 3-21. Twenty-one days after STZ injection the rats were sacrificed...
2017: PloS One
https://www.readbyqxmd.com/read/28466615/angiographic-embolization-in-pediatric-abdominal-trauma
#17
Yechiel Sweed, Jonathan Singer-Jordan, Sorin Papura, Norman Loberant, Alon Yulevich
BACKGROUND: Trauma is the leading cause of childhood morbidity and mortality. Abdominal bleeding is one of the common causes of mortality due to trauma. Angiography and embolization are well recognized as the primary treatments in certain cases of acute traumatic hemorrhage in adults; however, evidence is lacking in the pediatric population. OBJECTIVES: To assess the safety and efficacy of transcatheter arterial embolization (TAE) for blunt and penetrating abdominal and pelvic trauma in the pediatric age group...
November 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28457953/ifn-%C3%AE-is-effective-for-treatment-of-minimal-residual-disease-in-patients-with-acute-leukemia-after-allogeneic-hematopoietic-stem-cell-transplantation-results-of-a-registry-study
#18
Xiao-Dong Mo, Xiao-Hui Zhang, Lan-Ping Xu, Yu Wang, Chen-Hua Yan, Huan Chen, Yu-Hong Chen, Wei Han, Feng-Rong Wang, Jing-Zhi Wang, Kai-Yan Liu, Xiao-Jun Huang
The efficacy of minimal residual disease (MRD)-directed IFN-α treatment was investigated in acute leukemia patients who were positive for MRD after allogeneic hematopoietic stem cell transplantation (allo-HSCT) (n = 107). MRD-positive status was defined as positivity for leukemia-associated aberrant immune phenotypes or positivity for Wilms' tumor gene 1 in a single bone marrow sample. Recombinant human IFN-α-2b injections were administered subcutaneously 2 to 3 times per week for 6 months. The 2-year cumulative incidence of severe acute and chronic graft-versus-host disease after IFN-α treatment was 5...
April 27, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28454430/wt1-associated-protein-is-a-novel-prognostic-factor-in-pancreatic-ductal-adenocarcinoma
#19
Bing-Qi Li, Shuai Huang, Qian-Qian Shao, Jian Sun, Li Zhou, Lei You, Tai-Ping Zhang, Quan Liao, Jun-Chao Guo, Yu-Pei Zhao
Although Wilms tumor 1 (WT1)-associated protein (WTAP) was initially found to be a specific WT1-binding protein, it has increasingly attracted attention because of its oncogenic role in various types of malignancies, including cholangiocarcinoma, glioblastoma and acute myeloid leukemia. However, the clinical impact of WTAP on pancreatic ductal adenocarcinoma (PDAC) is still unknown. A total of 145 patients who underwent surgical treatment from 2004 to 2008 were enrolled in the present study. The cytoplasmic and nuclear expression of WTAP in tumor and adjacent normal tissues was examined by immunohistochemical analysis in order to investigate the relationship between WTAP and the clinicopathological factors and prognosis of patients with PDAC...
April 2017: Oncology Letters
https://www.readbyqxmd.com/read/28449403/effects-of-malnutrition-on-treatment-related-morbidity-and-survival-of-children-with-cancer-in-nicaragua
#20
Allison K Pribnow, Roberta Ortiz, Luis Fulgencio Báez, Luvy Mendieta, Sandra Luna-Fineman
BACKGROUND: Most children with cancer live in resource-limited countries where malnutrition is often prevalent. We identified the relationship between malnutrition and treatment-related morbidity (TRM), abandonment of therapy, and survival of children with cancer in Nicaragua to better inform targeted nutritional interventions. PROCEDURE: We conducted a retrospective review of patients aged 6 months to 18 years with newly diagnosed acute lymphoblastic leukemia, acute myeloid leukemia (AML), Wilms tumor, Hodgkin lymphoma, or Burkitt lymphoma (BL) who were treated between January 1, 2004, and December 31, 2007 at Children's Hospital Manuel de Jesus Rivera in Managua, Nicaragua...
April 27, 2017: Pediatric Blood & Cancer
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