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wilms tumors

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https://www.readbyqxmd.com/read/29138824/genetic-mutational-testing-of-chinese-children-with-familial-hematuria-with-biopsy%C3%A2-proven-fsgs
#1
Yongzhen Li, Ying Wang, Qingnan He, Xiqiang Dang, Yan Cao, Xiaochuan Wu, Shuanghong Mo, Xiaoxie He, Zhuwen Yi
Focal segmental glomerulosclerosis (FSGS) is a pathological lesion rather than a disease, with a diverse etiology. FSGS may result from genetic and non‑genetic factors. FSGS is considered a podocyte disease due to the fact that in the majority of patients with proven‑FSGS, the lesion results from defects in the podocyte structure or function. However, FSGS does not result exclusively from podocyte‑associated genes, however also from other genes including collagen IV‑associated genes. Patients who carry the collagen type IVA3 chain (COL4A3) or COL4A4 mutations usually exhibit Alport Syndrome (AS), thin basement membrane neuropathy or familial hematuria (FH)...
November 10, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29136988/renal-function-of-patients-with-synchronous-bilateral-wilms-tumor
#2
Denis A Cozzi, Silvia Ceccanti, Francesco Cozzi
No abstract text is available yet for this article.
December 2017: Annals of Surgery
https://www.readbyqxmd.com/read/29136698/-epithelial-predominant-adult-wilms-tumor-report-of-2-cases
#3
H F Zhang, X Wen, S S Wu, G P Ren
No abstract text is available yet for this article.
November 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29133167/patterns-of-lymph-node-sampling-and-the-impact-of-lymph-node-density-in-favorable-histology-wilms-tumor-an-analysis-of-the-national-cancer-database
#4
A F Saltzman, A Carrasco, A Amini, J H Aldrink, R Dasgupta, K W Gow, R D Glick, P F Ehrlich, N G Cost
INTRODUCTION: There is controversy about the role of lymph node (LN) sampling or dissection in the management of favorable histology (FH) Wilms tumor (WT), specifically how it performed and how it may impact survival. OBJECTIVE: The objective of this study was to analyze factors affecting LN sampling patterns and the impact of LN yield and density (number of positive LNs/LNs examined) on overall survival (OS) in patients with advanced-stage favorable histology Wilms tumor (FHWT)...
October 31, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29130032/successful-treatment-of-a-solitary-skull-metastasis-in-a-child-with-wilms-tumor
#5
Lucas A McDuffie, Robert J Fallon, Terry A Vik, Deborah F Billmire
This report presents the successful treatment of a child with a solitary metastatic lesion to the calvarium following treatment for Stage III anaplastic Wilms' Tumor.
July 2017: Journal of Pediatric Surgery Case Reports
https://www.readbyqxmd.com/read/29119252/image-based-surgical-risk-factors-for-wilms-tumor
#6
Takaharu Oue, Akihiro Yoneda, Noriaki Usui, Takashi Sasaki, Masahiro Zenitani, Natsumi Tanaka, Shuichiro Uehara, Soji Ibuka, Yuichi Takama, Hiroomi Okuyama
PURPOSE: The standard treatment for Wilms tumor (WT) is primary resection. However, in cases with unresectable tumor or tumor spillage, which are considered to have high surgical risks, more intensive chemotherapy and radiotherapy are required. In the present study, we retrospectively analyzed preoperative image parameters to identify factors associated with surgical risks. METHODS: Twenty-nine patients with WT were enrolled in this study. Data on various preoperative image parameters, such as tumor size, tumor volume, displacement of great vessels, and contralateral extension of the tumor were collected, and their relationship with surgical factors, including operative time, hemorrhage, tumor spillage, and unresectability were analyzed...
November 8, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29109067/the-insertion-in-the-double-stranded-rna-binding-domain-of-human-drosha-is-important-for-its-function
#7
Xiaoxiao Zhang, Peng Li, Jian Lin, Haochu Huang, Bin Yin, Yan Zeng
microRNAs (miRNAs) are first transcribed as long, primary transcripts, which are then processed by multiple enzymes and proteins to generate the single-stranded, approximately 22-nucleotide (nt)-long mature miRNAs. A critical step in animal miRNA biogenesis is the cleavage of primary miRNA transcripts (pri-miRNAs) to produce precursor miRNAs (pre-miRNAs) by the enzyme Drosha. How Drosha recognizes its substrates remains incompletely understood. In this study we constructed a series of human Drosha mutants and examined their enzymatic activities and interaction with RNAs...
November 3, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29100432/cancer-antigen-profiling-for-malignant-pleural-mesothelioma-immunotherapy-expression-and-coexpression-of-mesothelin-cancer-antigen-125-and-wilms-tumor-1
#8
Takashi Eguchi, Kyuichi Kadota, Marissa Mayor, Marjorie G Zauderer, Andreas Rimner, Valerie W Rusch, William D Travis, Michel Sadelain, Prasad S Adusumilli
Background: To develop cancer antigen-targeted immunotherapeutic strategies for malignant pleural mesothelioma (MPM), we investigated the individual and coexpressions of the cancer-associated antigens mesothelin (MSLN), cancer antigen 125 (CA125), and Wilms tumor 1 (WT1) in both epithelioid and non-epithelioid MPM. Methods: All available hematoxylin and eosin-stained slides from patients who were diagnosed with MPM (1989-2010) were reviewed. We constructed tissue microarrays from 283 patients (epithelioid = 234; non-epithelioid = 49)...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29096332/high-prognostic-value-of-pre-allogeneic-stem-cell-transplantation-minimal-residual-disease-detection-by-wt1-gene-expression-in-aml-transplanted-in-cytologic-complete-remission
#9
Anna Candoni, Federico De Marchi, Maria Elena Zannier, Davide Lazzarotto, Carla Filì, Maria Vittoria Dubbini, Nicholas Rabassi, Eleonora Toffoletti, Bonnie W Lau, Renato Fanin
We analyzed the outcome of allogeneic stem cell transplantation (allo-SCT) in acute myeloid leukemia (AML) patients according to molecular Minimal Residual Disease (MRD) status prior to allo-SCT. MRD was assessed by the quantitative expression of the pan-leukemic marker Wilms' tumor (WT1) gene, according to the validated LeukemiaNet method. Between 2005 and 2016, 122 consecutive AML patients, WT1 positive at diagnosis, received allo-SCT in cytologic complete remission (cCR). The median age at SCT was 53 years (range 18-70)...
October 27, 2017: Leukemia Research
https://www.readbyqxmd.com/read/29094095/wilms-tumor-treatment-outcomes-perspectives-from-a-low-income-setting
#10
Festus Njuguna, Hugo A Martijn, Robert Tenge Kuremu, Peter Saula, Patel Kirtika, Gilbert Olbara, Sandra Langat, Steve Martin, Jodi Skiles, Terry Vik, Gertjan J L Kaspers, Saskia Mostert
Purpose: Wilms tumor is the commonest renal malignancy in childhood. Survival in high-income countries is approximately 90%, whereas in low-income countries, it is less than 50%. This study assessed treatment outcomes of patients with Wilms tumor at a Kenyan academic hospital. Patients and Methods: We conducted a retrospective medical record review of all children diagnosed with Wilms tumor between 2010 and 2012. Data on treatment outcomes and various sociodemographic and clinical characteristics were collected...
October 2017: Journal of Global Oncology
https://www.readbyqxmd.com/read/29077255/irinotecan-for-relapsed-wilms-tumor-in-pediatric-patients-siop-experience-and-review-of-the-literature-a-report-from-the-siop-renal-tumor-study-group
#11
Janna A Hol, Marry M van den Heuvel-Eibrink, Norbert Graf, Kathy Pritchard-Jones, Jesper Brok, Harm van Tinteren, Lisa Howell, Arnauld Verschuur, Christophe Bergeron, Leo Kager, Serena Catania, Filippo Spreafico, Annelies M C Mavinkurve-Groothuis
While irinotecan has been studied in various pediatric solid tumors, its potential role in Wilms tumor (WT) is less clear. We evaluated response and outcome of irinotecan-containing regimens in relapsed WT and compared our results to the available literature. Among 14 evaluable patients, one complete response (CR) and two partial responses (PRs) were observed in patients with initial intermediate-risk (CR and PR) and blastemal-type histologies (PR). Two patients were alive at last follow-up showing no evidence of disease...
October 27, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29074135/evaluation-of-effect-of-preoperative-chemotherapy-on-wilms-tumor-histopathology
#12
Seppo Taskinen, Jouko Lohi, Minna Koskenvuo, Mervi Taskinen
PURPOSE: To evaluate usefulness of cutting needle biopsy (CNB) to recognize pediatric renal tumors and to predict the evolution of histology during preoperative chemotherapy of Wilms tumors. METHODS: Ninety pediatric patients were operated for renal tumors at our institution in 1988-2015. We included all 64 patients who had undergone CNB at diagnosis and whose CNB and nephrectomy samples were available for re-evaluation. RESULTS: The CNB was diagnostic in all 59 Wilms tumors but only in two out of five non-Wilms tumors...
October 6, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29073290/nutritional-status-at-the-moment-of-diagnosis-in-childhood-cancer-patients
#13
Joanna Połubok, Anna Malczewska, Małgorzata Rąpała, Jerzy Szymocha, Marta Kozicka, Katarzyna Dubieńska, Monika Duczek, Bernarda Kazanowska, Ewa Barg
INTRODUCTION: Children with a neoplastic disease are highly susceptible to malnutrition. The main objective of the study was to assess the frequency of undernourishment and obesity at the time of the diagnosis of the neoplastic disease at children. MATERIALS AND METHODS: The study included 734 patients (58% males) at the age 1-20,25, with the diagnosis of neoplasm in the years 1986-2014. The patients were divided into groups depending on the type of the diagnosis: 1) ALL, 2) ANLL, 3) HL, 4) NHL, 5) NBL, 6) Wilms tumor, 7) mesenchymal malignant tumor...
2017: Pediatric Endocrinology, Diabetes, and Metabolism
https://www.readbyqxmd.com/read/29070431/cytoreductive-surgery-crs-and-hyperthermic-intraperitoneal-chemotherapy-hipec-for-disseminated-intraabdominal-malignancies-in-children-a-single-institution-experience
#14
Osnat Zmora, Andrea Hayes-Jordan, Aviram Nissan, Iris Kventsel, Yoram Newmann, Kira Itskovsky, Shifra Ash, Sarina Levy-Mendelovich, Daniel Shinhar, Almog Ben-Yaakov, Amos Toren, Ron Bilik
PURPOSE: Our purpose was to present our institutional experience with performing complete cytoreduction surgery and heated intraoperative chemotherapy (CRS-HIPEC) for children with disseminated intraabdominal malignancies, guided by a leading international center performing CRS-HIPEC in children. METHODS: Retrospective chart review of all cases of CRS-HIPEC in children in our institution, examining diagnosis, preoperative management, operative management, postoperative treatment, short term outcome including length of stay and complications, and long term outcome including survival and recurrence of disease...
October 2, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29061165/sustained-endocrine-profiles-of-a-girl-with-wagr-syndrome
#15
Yui Takada, Yasunari Sakai, Yuki Matsushita, Kazuhiro Ohkubo, Yuhki Koga, Satoshi Akamine, Michiko Torio, Yoshito Ishizaki, Masafumi Sanefuji, Hiroyuki Torisu, Chad A Shaw, Masayo Kagami, Toshiro Hara, Shouichi Ohga
BACKGROUND: Wilms tumor, aniridia, genitourinary anomalies and mental retardation (WAGR) syndrome is a rare genetic disorder caused by heterozygous deletions of WT1 and PAX6 at chromosome 11p13. Deletion of BDNF is known eto be associated with hyperphagia and obesity in both humans and animal models; however, neuroendocrine and epigenetic profiles of individuals with WAGR syndrome remain to be determined. CASE PRESENTATION: We report a 5-year-old girl with the typical phenotype of WAGR syndrome...
October 23, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/29050111/-a-long-term-follow-up-report-of-pediatric-relapsed-wilms-tumor-after-retreatment
#16
T Y Wang, C Pan, Y J Gao, W T Hu, Q D Ye, M Zhou, J Y Tang
Objective: To investigate the long-term efficacy and prognostic factors of pediatric relapsed Wilms tumor (WT) after retreatment. Method: Sixteen children in Shanghai Children's Medical Center with relapsed Wilms tumor were enrolled consecutively in this study between April 2006 and June 2016. All patients were diagnosed according to pathology, imaging and medical and surgical oncologist's assistance. Relapse treatment included surgical excision, chemotherapy and selective radiation therapy. The clinical features, long-term outcomes and prognostic factors of patients were analyzed retrospectively...
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29042436/wilms-tumor-protein-dependent-transcription-of-vegf-receptor-2-and-hypoxia-regulate-expression-of-the-testis-promoting-gene-sox9-in-murine-embryonic-gonads
#17
Karin M Kirschner, Lina K Sciesielski, Katharina Krueger, Holger Scholz
The Wilms tumor protein 1 (WT1) has been implicated in the control of several genes in sexual development, but its function in gonad formation is still unclear. Here, we report that WT1 stimulates expression of Kdr, the gene encoding VEGF receptor 2, in murine embryonic gonads. We found that WT1 and KDR are co-expressed in Sertoli cells of the testes and somatic cells of embryonic ovaries. Vivo-morpholino-mediated WT1 knockdown decreased Kdr transcripts in cultured embryonic gonads at multiple developmental stages...
October 17, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29041012/wilms-tumor-gene-1-wt1-peptide-vaccine-therapy-for-hematological-malignancies-from-ctl-epitope-identification-to-recent-progress-in-clinical-studies-including-a-cure-oriented-strategy
#18
Yoshihiro Oka, Akihiro Tsuboi, Jun Nakata, Sumiyuki Nishida, Naoki Hosen, Atsushi Kumanogoh, Yusuke Oji, Haruo Sugiyama
The identification of human Wilms' tumor gene 1 (WT1) protein-derived cytotoxic T lymphocyte (CTL) epitopes and the in vivo efficacy of WT1 peptide-based immunotherapy in a mouse model were reported in 2000. This successful basic research led to clinical studies of a WT1 peptide vaccine, and a positive impact on clinical response was first demonstrated in 2003 in the form of a reduction in blast cells of vaccine-treated patients with myelodysplastic syndromes (MDS). Since then, data on WT1 peptide vaccine-treated patients with immunological and/or clinical response have been accumulated...
2017: Oncology Research and Treatment
https://www.readbyqxmd.com/read/29040381/the-cug-translated-wt1-not-aug-wt1-is-an-oncogene
#19
Kun Yeong Lee, Young Jin Jeon, Hong Gyum Kim, Joohyun Ryu, Do Young Lim, Sung Keun Jung, Dong Hoon Yu, Hanyong Chen, Ann M Bode, Zigang Dong
The Wilms' tumor 1 (WT1) gene is believed to act as a canonical tumor suppressor. However, it has also been reported to function as an oncogene. Germline WT1 deletion is associated with Wilms' tumor and exogenous WT1 cDNA introduction into cells induces the transcriptional suppression of its oncogenic target genes. In contrast, high WT1 expression is associated with poor prognosis in patients with various cancers. Why WT1 acts as a tumor suppressor under certain conditions, but as an oncogene under other conditions is unknown...
October 10, 2017: Carcinogenesis
https://www.readbyqxmd.com/read/29038164/pka-creb-signaling-prevents-adriamycin-induced-podocyte-apoptosis-via-upregulation-of-mitochondrial-respiratory-chain-complexes
#20
Kewei Xie, Mingli Zhu, Peng Xiang, Xiaohuan Chen, Ayijiaken Kasimumali, Renhua Lu, Qin Wang, Shan Mou, Zhaohui Ni, Leyi Gu, Huihua Pang
Previous work showed that the activation of protein kinase A (PKA) signaling promoted mitochondrial fusion and prevented podocyte apoptosis. The cAMP response element binding protein (CREB) is the main downstream transcription factor of PKA signaling. Herein, we show that the pKA-agonist pCPT-cAMP prevented the production of adriamycin (ADR)-induced reactive oxygen species and apoptosis in podocytes, which were inhibited by CREB RNAi. The activation of PKA enhanced mitochondrial function and prevented the ADR-induced decrease of mitochondrial respiratory chain complex I subunits, NADH-ubiquinone oxidoreductase complex (ND) 1/3/4 genes, and protein expression...
October 16, 2017: Molecular and Cellular Biology
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