keyword
MENU ▼
Read by QxMD icon Read
search

Wilm tumor 1

keyword
https://www.readbyqxmd.com/read/28081536/wt1-haploinsufficiency-supports-milder-renal-manifestation-in-two-patients-with-denys-drash-syndrome
#1
Mara S Guaragna, Juliana G Ribeiro de Andrade, Bárbara de Freitas Carli, Vera M S Belangero, Andréa T Maciel-Guerra, Gil Guerra-Júnior, Maricilda P de Mello
Denys-Drash syndrome (DDS) is characterized by nephropathy, genital abnormalities, and predisposition to Wilms' tumor. DDS patients usually present heterozygous de novo germline WT1 mutations. The WT1 gene comprises 10 exons encoding the N-terminal transactivation and the C-terminal DNA-binding regions. Two unrelated patients with genital ambiguity and Wilms' tumor were analyzed by sequencing of the WT1 gene, and 3 mutations in exon 1 were identified of which 2 are novel. Patient 1 carried a c.555delC mutation that causes a frameshift and a premature stop codon...
January 13, 2017: Sexual Development: Genetics, Molecular Biology, Evolution, Endocrinology, Embryology, and Pathology of Sex Determination and Differentiation
https://www.readbyqxmd.com/read/28072975/-molecular-features-of-metanephric-adenoma-and-their-values-in-differential-diagnosis
#2
X Wang, S S Shi, W R Yang, S B Ye, R Li, H H Ma, R S Zhang, Z F Lu, X J Zhou, Q Rao
Objective: To study the molecular features of metanephric adenoma (MA) and discuss their values in differential diagnosis. Methods: BRAF V600E immunohistochemistry (IHC) using the mutation-specific VE1 monoclonal antibody and Sanger sequencing of BRAF mutations were performed on 21 MAs, 16 epithelial-predominant Wilms tumors (e-WT) and 20 the solid variant of papillary renal cell carcinomas (s-PRCC) respectively. p16 protein was detected by IHC also. Fluorescence in situ hybridization (FISH) analyses using centromeric probes for chromosome 7 and 17 were performed on the three renal tumors in parallel...
January 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28068926/screening-of-wt1-mutations-in-exon-8-and-9-in-children-with-steroid-resistant-nephrotic-syndrome-from-a-single-centre-and-establishment-of-a-rapid-screening-assay-using-high-resolution-melting-analysis-in-a-clinical-setting
#3
Annes Siji, Varsha Chhotusing Pardeshi, Shilpa Ravindran, Ambily Vasudevan, Anil Vasudevan
BACKGROUND: Mutations in Wilm's tumor 1 (WT1) gene is one of the commonly reported genetic mutations in children with steroid resistant nephrotic syndrome (SRNS). We report the results of direct sequencing of exons 8 and 9 of WT1 gene in 100 children with SRNS from a single centre. We standardized and validated High Resolution Melt (HRM) as a rapid and cost effective screening step to identify individuals with normal sequence and distinguish it from those with a potential mutation. Since only mutation positive samples identified by HRM will be further processed for sequencing it will help in reducing the sequencing burden and speed up the screening process...
January 10, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28067876/impact-of-wilms-tumor-1-expression-on-outcome-of-patients-undergoing-allogeneic-stem-cell-transplantation-for-aml
#4
R Duléry, O Nibourel, J Gauthier, V Elsermans, H Behal, V Coiteux, L Magro, A Renneville, A Marceau, T Boyer, B Quesnel, C Preudhomme, A Duhamel, I Yakoub-Agha
The monitoring of the minimal residual disease by Wilms' tumor 1 expression (MRD(WT1)) is a standardized test, which can be used in over 80% of patients with AML. To investigate the prognostic value of MRD(WT1) in patients undergoing allogeneic stem cell transplantation (allo-SCT) for AML, MRD(WT1) was monitored 3 months after transplantation in 139 patients. MRD(WT1) positivity did not lead to any therapeutic intervention. Median follow-up was 39.3 (6.4-99.8) months. Patients with positive MRD(WT1) at 3 months experienced more often post-transplant relapse (27/30, 90%) than those with negative MRD(WT1) (16/109, 14...
January 9, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28066808/effect-of-mr-imaging-contrast-thresholds-on-prediction-of-neoadjuvant-chemotherapy-response-in-breast-cancer-subtypes-a-subgroup-analysis-of-the-acrin-6657-i-spy-1-trial
#5
Wen Li, Vignesh Arasu, David C Newitt, Ella F Jones, Lisa Wilmes, Jessica Gibbs, John Kornak, Bonnie N Joe, Laura J Esserman, Nola M Hylton
Functional tumor volume (FTV) measurements by dynamic contrast-enhanced magnetic resonance imaging can predict treatment outcomes for women receiving neoadjuvant chemotherapy for breast cancer. Here, we explore whether the contrast thresholds used to define FTV could be adjusted by breast cancer subtype to improve predictive performance. Absolute FTV and percent change in FTV (ΔFTV) at sequential time-points during treatment were calculated and investigated as predictors of pathologic complete response at surgery...
December 2016: Tomography: a Journal for Imaging Research
https://www.readbyqxmd.com/read/28054314/turning-back-the-wheel-inducing-mesenchymal-to-epithelial-transition-via-wilms-tumor-1-knockdown-in-human-mesothelioma-cell-lines-to-influence-proliferation-invasiveness-and-chemotaxis
#6
Till Plönes, Mitja Fischer, Kerstin Höhne, Hiromi Sato, Joachim Müller-Quernheim, Gernot Zissel
Malignant pleural mesothelioma (MPM) is a highly aggressive tumor that arises from the surface of the pleura and is associated with a history of asbestos exposure. The tumor is characterized by a strong local invasiveness and a poor response to any single modality therapy. Therefore clinical outcome of patients with MPM is poor and median survival time of untreated patients with MPM is 7 months from initial diagnosis. The Wilms Tumor Protein 1 (WT1) is a transcription factor which is highly expressed by MPM and is involved in cellular development and survival...
January 4, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28048032/su-f-j-63-abdominal-diameter-changes-in-children-during-volumetric-modulated-arc-therapy-vmat-is-re-planning-needed
#7
F Guerreiro, G Janssens, E Seravalli, B Raaymakers
PURPOSE: To investigate the dosimetric impact of daily changes in patient's diameter, due to weight gain/loss and air in the bowel, based on CBCT information during radiotherapy treatment of pediatric abdominal tumors. METHODS: 10 pediatric patients with neuroblastoma (n=6) and Wilms' (n=4) tumors were included. Available CBCTs were affinely registered to the planning CT for daily set-up variations corrections. A density override approach assigning air-density to the random air pockets and water-density to the remaining anatomy was used to determine the CBCT and CT dose...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28047673/su-d-207a-06-pediatric-abdominal-organ-motion-quantified-via-a-novel-4d-mri-method
#8
J Uh, M J Krasin, J T Lucas, C Tinkle, T E Merchant, C Hua
PURPOSE: To develop a 4D MRI method for assessing respiration-induced abdominal organ motion in children receiving radiation therapy. METHODS: A 4D MRI using internal image-based respiratory surrogate has been developed and implemented on a clinical scanner (1.5T Siemens Avanto). Ten patients (younger group: N=6, 2-5 years, anesthetized; older group: N=4, 11-15 years) with neuroblastoma, Wilm's tumor rhabdomyosarcoma, or desmoplastic small round cell tumor received free breathing 4D MRI scans for treatment planning...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/27997722/pitavastatin-suppresses-hyperglycemia-induced-podocyte-injury-via-bone-morphogenetic-protein-7-preservation
#9
Makoto Ohigashi, Miyuki Kobara, Tamotsu Takahashi, Hiroe Toba, Takehiko Wada, Tetsuo Nakata
BACKGROUND: Podocytes form the essential components of the glomerular filtration barrier and play a critical role in diabetic nephropathy. Recent evidence suggests that HMG-CoA reductase inhibitors (statins) exert renoprotective effects. We investigated whether pitavastatin directly suppresses hyperglycemia-induced podocyte injury using cultured podocytes and, if so, the mechanism of the beneficial effects. METHODS AND RESULTS: Cultured podocytes were exposed to media containing normal (NG; 5 mmol/L) or high (HG; 25 mmol/L) glucose for one week...
December 20, 2016: Clinical and Experimental Pharmacology & Physiology
https://www.readbyqxmd.com/read/27993090/wilms-tumor-1-wt1-targeted-cancer-vaccines-to-extend-survival-for-patients-with-pancreatic-cancer
#10
Shigeo Koido, Masato Okamoto, Shigetaka Shimodaira, Haruo Sugiyama
Despite novel chemotherapy treatments, pancreatic ductal adenocarcinoma (PDA) remains a lethal disease. New targeted cancer vaccines may represent a viable option for patients with PDA. The Wilms' tumor 1 (WT1) antigen is one of the most widely expressed tumor-associated antigens in various types of tumors, including PDA. Recent reports have indicated that WT1-targeted cancer vaccines for patients with PDA mediated a potent antitumor effect when combined with chemotherapy in preclinical and clinical studies...
November 2016: Immunotherapy
https://www.readbyqxmd.com/read/27990711/a-novel-functional-variant-in-wilms-tumor-1-wt1-is-associated-with-idiopathic-non-obstructive-azoospermia
#11
Jin Xu, Lile Jiang, Wenzhu Yu, Haibin Guo, Helong Zhang, Duo Wei, Lingling Liang, Ke Feng, Xiaobing Song, Qi Liu, Bingbing Song, Haoying Hao, Ying Zhang, Cuilian Zhang
Idiopathic non-obstructive azoospermia (INOA) is one of the most severe forms of male infertility, yet its pathophysiology remains unclear. WT1 (Wilm's tumor 1) regulates the polarity of Sertoli cells, thereby playing a critical, indirect role in spermatogenesis. Here, we evaluated WT1 gene variation associates with INOA by assessing its promoter and coding regions in 200 patients diagnosed with INOA and 200 proven fertile men. Three novel variants in the WT1 coding region were detected only in INOA patients, including two synonymous variants and one missense variant, p...
December 19, 2016: Molecular Reproduction and Development
https://www.readbyqxmd.com/read/27977403/clinical-and-molecular-characterization-of-beckwith-wiedemann-syndrome-in-a-chinese-population
#12
Ho Ming Luk
BACKGROUND: The objective of this study was to examine the clinical and molecular features, genotype-phenotype correlation and the efficacy of different diagnostic criteria for predicting a positive molecular test in Chinese Beckwith-Wiedemann syndrome (BWS) patients. METHODS: A retrospective tertiary-wide study was performed in Hong Kong with 27 molecularly confirmed BWS patients between January 2010 and September 2015. RESULTS: It was observed that 48...
January 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27960539/chemokine-receptor-cxcr4-plays-a-crucial-role-in-mediating-oxidative-stress-induced-podocyte-injury
#13
Hongyan Mo, Qinyu Wu, Jinhua Miao, Congwei Luo, Xue Hong, Yongping Wang, Lan Tang, Fan Fan Hou, Youhua Liu, Lili Zhou
AIMS: Oxidative stress plays a role in mediating podocyte injury and proteinuria. However, the underlying mechanism remains poorly understood. In this study, we investigated the potential role of CXCR4, the chemokine receptor for SDF-1α, in mediating oxidative stress-induced podocyte injury. RESULTS: In mouse model of adriamycin nephropathy (ADR), CXCR4 expression was significantly induced in podocytes as early as 3 days. This was accompanied by an increased upregulation of oxidative stress in podocyte, as demonstrated by MDA assay, nitrotyrosine staining and secretion of 8-OHdG in urine, and induction of NOX2 and NOX4, major subunits of NADPH oxidase...
December 13, 2016: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/27941286/adoptive-immunotherapy-utilizing-cancer-antigen-specific-t-cell-receptors
#14
Kazushi Tanimoto, Hiroshi Fujiwara
Synthetic immunology based on rapidly-advancing gene-engineering and immunobiology has made novel anticancer adoptive immunotherapies, using gene-modified T lymphocytes to express cancer antigen-specific receptors, a reality. Various technological innovations have overcome recent difficulties and achieved clear and long-lasting clinical efficacy against tumors, while seeking more powerful effector gene-modified T cells has yielded serious treatment-related adverse events. In this article, along with introducing our clinical trial for a novel anti-leukemia adoptive immunotherapy regimen using gene-modified autologous lymphocytes to express leukemia antigen Wilms Tumor 1(WT1)-specific T cell receptor (TCR) against refractory acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (MDS), we provide an overview of the current status of this emerging treatment option and discuss its future form in the context of neoantigens encoded by mutated genes in cancer cells and immune checkpoint inhibitors...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27934559/recurrent-nephrotic-syndrome-after-renal-transplant-in-children
#15
Bassam Saeed, Huda Mazloum
OBJECTIVES: Recurrent disease occurs in around 30% of children transplanted for steroid-resistant nephrotic syndrome. Its precipitating risk factors have rarely been studied in the Middle East. The aim of our study was to determine what characterizes posttransplant recurrence of nephrotic syndrome in Syrian children. MATERIALS AND METHODS: We performed a retrospective analysis of 12 nephrotic children who received 1 renal allograft at the Kidney Hospital in Damascus from 2002 to 2013...
December 2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/27924074/optimized-t-cell-receptor-mimic-chimeric-antigen-receptor-t-cells-directed-toward-the-intracellular-wilms-tumor-1-antigen
#16
S Rafiq, T J Purdon, A F Daniyan, M Koneru, T Dao, C Liu, D A Scheinberg, R J Brentjens
CD19-directed chimeric antigen receptor (CAR) T cells are clinically effective in a limited set of leukemia patients. However, CAR T-cell therapy thus far has been largely restricted to targeting extracellular tumor-associated antigens (TAA). Herein, we report a T-cell receptor-mimic (TCRm) CAR, termed WT1-28z, that is reactive to a peptide portion of the intracellular onco-protein Wilms Tumor 1(WT1), as it is expressed on the surface of the tumor cell in the context of HLA-A*02:01. T cells modified to express WT1-28z specifically targeted and lysed HLA-A*02:01+ WT1+ tumors and enhanced survival of mice engrafted with HLA-A*02:01+, WT1+ leukemia or ovarian tumors...
January 3, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/27922671/differential-detection-of-cytoplasmic-wilms-tumor-1-expression-by-immunohistochemistry-western-blotting-and-mrna-quantification
#17
Takehiro Maki, Hiroaki Ikeda, Aki Kuroda, Noriaki Kyogoku, Yoshiyuki Yamamura, Yukiko Tabata, Takehiro Abiko, Takahiro Tsuchikawa, Yasuhiro Hida, Toshiaki Shichinohe, Eiichi Tanaka, Kichizo Kaga, Kanako Hatanaka, Yoshihiro Matsuno, Naoko Imai, Satoshi Hirano
Wilms tumor 1 (WT1) is considered to be a promising target of cancer treatment because it has been reported to be frequently expressed at high levels in various malignancies. Although WT1-targeted cancer treatment has been initiated, conclusive detection methods for WT1 are not established. The present study aimed to consolidate immunohistochemistry for WT1 with statistical basis. Transfected cells with forced WT1 expression yielded specific western blot bands and nuclear immunostaining; cytoplasmic immunostaining was not specifically recognized...
January 2017: International Journal of Oncology
https://www.readbyqxmd.com/read/27911068/-laparoscopic-nephron-sparing-surgery-for-wilms-tumor-description-of-two-cases
#18
L López, M Copete, P Villamizar
Surgical resection is the mainstay of treatment for Wilms tumor. The research progress of the large study groups worldwide has reduced mortality. However, searching to minimize morbidity, the minimally invasive approach has been applied in selected patients. This article describes two cases of Wilms tumor managed with laparoscopic nephron sparing surgery. The case 1 with Beckwith-Wiedemann syndrome and bilateral disease, and the case 2 non-syndromic unilateral. The approach was intraperitoneally, without intraoperative complications...
January 25, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27907819/giant-primary-malignant-mesothelioma-of-the-liver-a-case-report
#19
Ruba Haji Ali, Mohamad Khalife, Ghina El Nounou, Ruba Zuhri Yafi, Hussein Nassar, Zeinab Aidibe, Randa Raad, Rania Abou Eid, Walid Faraj
INTRODUCTION: Malignant mesothelioma is a rare neoplasm of mesothelial cells arising most frequently in the pleura or peritoneum and less frequently in the liver. CASE PRESENTATION: We present a case of primary hepatic mesothelioma of 41year old woman. She had no history of asbestos exposure or cancer. Abdominal computed tomography (CT) showed 21cm intrahepatic mass in the right lobe with many cystic lesions and few small calcifications. Pathology showed a biphasic cellular pattern...
November 11, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27904661/a-novel-sphingosine-kinase-1-inhibitor-ski-5c-induces-cell-death-of-wilms-tumor-cells-in-vitro-and-in-vivo
#20
Zhi-Heng Li, Yan-Fang Tao, Li-Xiao Xu, He Zhao, Xiao-Lu Li, Fang Fang, Yi Wu, Jun Lu, Yan-Hong Li, Wei-Wei Du, Jun-Li Ren, Yi-Ping Li, Yun-Yun Xu, Xing Feng, Jian Wang, Wei-Qi He, Jian Pan
Sphingosine kinase 1 (SphK1) is over-expressed in many cancers and therefore serves as a biomarker for cancer prognosis. SKI-5C is a new SphK1 inhibitor, and until now its molecular function in Wilms' tumor cells remained unknown. Here, using CCK-8 and nude mice experiments we assessed cell growth in Wilms' tumor cell lines (SK-NEP-1 and G401) in vitro and in vivo. We demonstrated that SphK1 is highly expressed in SK-NEP-1 and G401 cells, and through annexin V/propidium iodide staining and flow cytometry analysis, we detected cell apoptosis...
2016: American Journal of Translational Research
keyword
keyword
46778
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"