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https://www.readbyqxmd.com/read/29753838/wilms-tumor-1-wt1-expression-using-a-standardized-european-leukemia-net-certified-assay-compared-to-other-methods-for-detection-of-minimal-residual-disease-in-mds-and-aml-patients-after-allogeneic-blood-stem-cell-transplantation
#1
Christina Rautenberg, Sabrina Pechtel, Barbara Hildebrandt, Beate Betz, Ariane Dienst, Kathrin Nachtkamp, Mustafa Kondakci, Stefanie Geyh, Dagmar Wieczorek, Rainer Haas, Ulrich Germing, Guido Kobbe, Thomas Schroeder
Overexpressed Wilms' Tumor 1 (WT1) gene is informative in many patients with acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS) and is measurable in peripheral blood (PB). Despite these advantages WT1 has not broadly been established as marker for minimal residual disease (MRD) monitoring after allogeneic transplantation (allo-HSCT) due to limited patient numbers, differing sample sources and non-standardized in-house methods. To estimate its value as MRD marker we serially quantified PB WT1 expression using a standardized European Leukemia Net-certified assay in 59 patients with AML and MDS after allo-HSCT...
May 10, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29751751/primary-synovial-sarcoma-of-the-kidney-a-case-report-of-complete-pathological-response-at-a-lebanese-tertiary-care-center
#2
Alissar El Chediak, Deborah Mukherji, Sally Temraz, Samer Nassif, Sara Sinno, Rami Mahfouz, Ali Shamseddine
BACKGROUND: Primary synovial sarcoma of the kidney is a rare type of soft tissue sarcoma. Its presenting features can resemble those of other renal tumors; rendering its early diagnosis, a dilemma. Several cases of renal synovial sarcoma have been reported in the literature with varying treatment options and outcomes. This article describes a rare case of primary renal synovial sarcoma and reviews all cases in the literature. CASE PRESENTATION: A 26-year-old male presented with flank pain and hematuria...
May 11, 2018: BMC Urology
https://www.readbyqxmd.com/read/29739109/a-proposed-kinetic-model-for-the-diagnostic-and-prognostic-value-of-wt1-and-p53-in-acute-myeloid-leukemia
#3
Mohammad A Bani-Ahmad, Suleimman A Al-Sweedan, Mohammad A Al-Asseiri, Ahed J Alkhatib
BACKGROUND: Wilms tumor (WT1) and p53 proteins were identified in the pathogenesis of several malignancies, including hematological malignancies. As a result of their interaction and diverse context-specific functions, this study aimed to emphasize the diagnostic and prognostic impacts of WT1 and p53 expression in acute myeloid leukemia (AML). METHODS: Twelve bone marrow (BM) biopsies were obtained from AML patients who were diagnosed in accordance with the French-American-British diagnostic criteria...
March 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29733801/evaluation-of-renal-tumors-in-children
#4
Gülçin Bozlu, Elvan Çağlar Çıtak
OBJECTIVE: Renal tumors are not uncommon in children. In this study, we aimed to evaluate the clinical and pathological features of renal tumors in children. MATERIAL AND METHODS: Between January 2008 and December 2017, the records of children with renal tumors in our institution were retrospectively analyzed. Data collected were composed of demographic and clinical characteristics including gender, age at time of diagnosis, symptoms, laterality of the tumor and pathological evaluation...
May 2018: Turkish Journal of Urology
https://www.readbyqxmd.com/read/29714464/insulin-like-growth-factor-1-receptor-expression-in-pediatric-tumors-a-comparative-immunohistochemical-study
#5
Resul Karakuş, Esra Karakuş, Suna Emir, Ayper Kaçar, Derya Özyörük
Background/aim: Insulin-like growth factor-1 receptor (IGF-1R) is a pivotal receptor tyrosine kinase involved in the cell cycle and malignant tumor transformation. It is differentially expressed in various types of tumors. We aimed to determine the expression of IGF- 1R in different pediatric tumors and to shed light on possible new indications of anti-IGF-1R treatment approaches. Materials and methods: A total of 147 specimens were analyzed according to their expression of IGF-1R. Specimens included those from rhabdomyosarcomas, Wilms tumors, Ewing sarcoma/primitive neuroectodermal tumors, peripheral neuroblastic tumors, acute lymphoblastic lymphoma, Hodgkin lymphoma, Burkitt lymphoma, retinoblastoma, pleuropulmonary blastoma, Langerhans cell histiocytosis, endodermal sinus tumors (ESTs), and myeloid sarcoma...
April 30, 2018: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29708625/role-of-the-wilms-tumor-suppressor-gene-wt1-in-pancreatic-development
#6
Laura Ariza, Ana Cañete, Anabel Rojas, Ramón Muñoz-Chápuli, Rita Carmona
The Wilms tumor suppressor gene (Wt1) encodes a transcription factor involved in the development of a number of organs, but the role played by Wt1 in pancreatic development is unknown. The pancreas contains a population of pancreatic stellate cells (PSC) very important for pancreatic physiology. We described elsewhere that hepatic stellate cells originate from the WT1-expressing liver mesothelium. Thus, we checked if the origin of PSCs was similar. WT1 is expressed in the pancreatic mesothelium. Between E10...
April 30, 2018: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/29704588/case-report-nephron-sparing-surgery-in-a-patient-with-bilateral-multifocal-wilms-tumor
#7
Diana K Bowen, Christopher J Long, Frank M Balis, Thomas F Kolon
We present a case of bilateral multifocal Wilms tumor in a non-syndromic 12 month old male. Our management approach included twelve weeks of preoperative chemotherapy for maximal tumor shrinkage and, despite the central location of the tumors, successful staged bilateral nephron-sparing surgery. We advocate for a broader application of nephron-sparing surgery in Wilms tumor cases with the goal of preserving renal function without compromising oncologic outcomes.
April 25, 2018: Urology
https://www.readbyqxmd.com/read/29700824/clinical-molecular-genetics-and-therapeutic-aspects-of-syndromic-obesity
#8
REVIEW
E Geets, M E C Meuwissen, W Van Hul
Obesity has become a major health problem worldwide. To date, more than 25 different syndromic forms of obesity are known in which one (monogenic) or multiple (polygenic) genes are involved. This review gives an overview of these forms and focuses more in detail on six syndromes: Prader Willi Syndrome and Prader Willi like phenotype, Bardet Biedl Syndrome, Alström Syndrome, Wilms tumor, Aniridia, Genitourinary malformations and mental Retardation syndrome and 16p11.2 (micro)deletions. Years of research provided plenty of information on the molecular genetics of these disorders and the obesity phenotype leading to a more individualized treatment of the symptoms, however, a lot of questions still remain unanswered...
April 26, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29698806/identification-of-two-14q32-deletions-involving-dicer1-associated-with-the-development-of-dicer1-related-tumors
#9
John C Herriges, Sara Brown, Maria Longhurst, Jillian Ozmore, John B Moeschler, Aura Janze, Jeanne Meck, Sarah T South, Erica F Andersen
DICER1 encodes an RNase III endonuclease protein that regulates the production of small non-coding RNAs. Germline mutations in DICER1 are associated with an autosomal dominant hereditary cancer predisposition syndrome that confers an increased risk for the development of several rare childhood and adult-onset tumors, the most frequent of which include pleuropulmonary blastoma, ovarian sex cord-stromal tumors, cystic nephroma, and thyroid gland neoplasia. The majority of reported germline DICER1 mutations are truncating sequence-level alterations, suggesting that a loss-of-function type mechanism drives tumor formation in DICER1 syndrome...
April 23, 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29693796/a-phase-1-study-of-cabozantinib-in-children-and-adolescents-with-recurrent-or-refractory-solid-tumors-including-cns-tumors-trial-advl1211-a-report-from-the-children-s-oncology-group
#10
Meredith K Chuk, Brigitte C Widemann, Charles G Minard, Xiaowei Liu, AeRang Kim, Melanie Brooke Bernhardt, Rachel A Kudgus, Joel M Reid, Stephan D Voss, Susan Blaney, Elizabeth Fox, Brenda J Weigel
BACKGROUND: We conducted a phase 1 trial to determine the maximum tolerated dose (MTD), toxicity profile, pharmacokinetics (PK), pharmacodynamics (PD), and preliminary activity of cabozantinib in children with refractory or relapsed solid tumors. METHODS: Patients received cabozantinib tablets on a continuous dosing schedule in a rolling-six escalating phase 1 trial design. PK and PD studies were performed. RESULTS: Forty-one patients, median (range) age 13 (4-18) years, received cabozantinib to achieve a weekly cumulative dose equivalent to 30 (n = 6), 40 (n = 23)...
April 25, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29689364/reversible-renal-glomerular-dysfunction-in-guinea-pigs-exposed-to-glutaraldehyde-polymerized-cell-free-hemoglobin
#11
Xiaoyuan Zhang, Matthew C Williams, Otgonchimeg Rentsendorj, Felice D'Agnillo
Chemically modified hemoglobin (Hb)-based oxygen carriers are promising oxygen replacement therapeutics however their potential renal effects are not fully understood. Using a guinea pig exchange transfusion model, we examined the effects of glutaraldehyde-polymerized bovine hemoglobin (HbG) on the permeability and integrity of the glomerular filtration barrier (GFB), which is comprised of podocytes, fenestrated endothelium, and the glomerular basement membrane. HbG induced marked proteinuria characterized in part by the loss of high molecular weight proteins, including albumin, immunoglobulin, and transferrin, at 4 and 12 h post-infusion that resolved by 72 h...
April 22, 2018: Toxicology
https://www.readbyqxmd.com/read/29686964/extensive-synchronous-bilateral-wilms-tumor-treated-with-nephron-sparing-surgery
#12
Laura DiChiacchio, Nicole M Shockcor, Regina Macatangay, Eric Strauch
No abstract text is available yet for this article.
May 2018: Urology Case Reports
https://www.readbyqxmd.com/read/29681695/use-of-pretherapy-core-biopsy-in-the-diagnosis-of-pediatric-renal-tumors
#13
Jujju Jacob Kurian, Kenneth R L Nongpiur, Susan Jehangir
Background: Pretreatment core biopsy of pediatric renal tumors has been advocated by United Kingdom Children's Cancer Study Group to circumvent the disadvantage of International Society of Paediatric Oncology protocol, where neoadjuvant chemotherapy initiated without histopathological confirmation can result in over- or under-treatment. Aim: This study aims (a) to assess if pretherapy core biopsy correlates with the nephrectomy biopsy; (b) to assess if neoadjuvant chemotherapy changes Wilms tumor (WT) histology, and (c) to assess the incidence of biopsy site recurrence...
April 2018: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/29680375/n-6-methyladenosine-mediates-the-cellular-proliferation-and-apoptosis-via-micrornas-in-arsenite-transformed-cells
#14
Shiyan Gu, Donglei Sun, Huangmei Dai, Zunzhen Zhang
N6 -methyladenosine (m6 A) modification is implicated to play an important role in cellular biological processes, but its regulatory mechanisms in arsenite-induced carcinogenesis are largely unknown. Here, human bronchial epithelial(HBE)cells were chronically treated with 2.5 μM arsenite sodium (NaAsO2 ) for about 13 weeks and these cells were identified with malignant phenotype which was demonstrated by increased levels of cellular proliferation, percentages of plate colony formation and soft agar clone formation, and high potential of resistance to apoptotic induction...
April 19, 2018: Toxicology Letters
https://www.readbyqxmd.com/read/29677881/re-risk-of-adverse-health-and-social-outcomes-up-to-50-years-after-wilms-tumor-the-british-childhood-cancer-survivor-study
#15
Douglas A Canning
No abstract text is available yet for this article.
May 2018: Journal of Urology
https://www.readbyqxmd.com/read/29670401/the-impact-of-the-lymph-node-density-on-overall-survival-in-patients-with-wilms-tumor-a-seer-analysis
#16
Haisheng You, Jin Yang, Qingqing Liu, Lina Tang, Qingting Bu, Zhenyu Pan, Jun Lyu
Objective: The objective of this study was to determine the impact of the lymph node density (LND) on overall survival of patients with Wilms' tumor (WT) using the Surveillance, Epidemiology, and End Results (SEER) database. Methods: Data from the SEER database were extracted from patients with WT in whom the LND could be obtained. Patients were divided into a low LND group and high LND group. Survival curves based on the LND stratification were plotted using the Kaplan-Meier method and compared with the log-rank test...
2018: Cancer Management and Research
https://www.readbyqxmd.com/read/29663071/cyclosporine-a-responsive-congenital-nephrotic-syndrome-with-single-heterozygous-variants-in-nphs1-nphs2-and-plce1
#17
Anna Eichinger, Sabine Ponsel, Carsten Bergmann, Roman Günthner, Julia Hoefele, Kerstin Amann, Bärbel Lange-Sperandio
BACKGROUND: Congenital nephrotic syndrome (CNS) is primarily a monogenetic disease, with the majority of cases due to changes in five different genes: the nephrin (NPHS1), podocin (NPHS2), Wilms tumor 1 (WT1), laminin ß2 (LAMB2), and phospholipase C epsilon 1 (PLCE1, NPHS3) gene. Usually CNS is not responsive to immunosuppressive therapy, but treatment with ACE inhibitors, AT1 receptor blockade and/or indomethacin can reduce proteinuria. If the disease progresses to end-stage renal disease, kidney transplantation is the therapy of choice...
April 16, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29662637/the-wilms-tumor-gene-1-is-a-prognostic-factor-in-myelodysplastic-syndrome-a-meta-analysis
#18
Yanan Jiang, Lin Liu, Jinhuan Wang, Zeng Cao, Zhigang Zhao
Previous studies have suggested that Wilms' tumor gene-1 (WT1) may be related to a decrease in both relapse-free survival (RFS) and overall survival (OS) for patients with myelodysplastic syndrome (MDS). Therefore, we conducted a meta-analysis on the utility of WT1 as a prognostic indicator of MDS. Published reports were searched in the following databases: Cochrane Library, PubMed, Embase, and Web of Science. The meta-analysis was conducted using the Cochrane Collaboration RevMan 5.2 software. Six publications with 450 total patients met the inclusion criteria and were subjected to further examination...
March 23, 2018: Oncotarget
https://www.readbyqxmd.com/read/29659330/treatment-of-stage-iv-favorable-histology-wilms-tumor-with-lung-metastases-a-report-from-the-children-s-oncology-group-aren0533-study
#19
David B Dix, Nita L Seibel, Yueh-Yun Chi, Geetika Khanna, Eric Gratias, James R Anderson, Elizabeth A Mullen, James I Geller, John A Kalapurakal, Arnold C Paulino, Elizabeth J Perlman, Peter F Ehrlich, Marcio Malogolowkin, Julie M Gastier-Foster, Elizabeth Wagner, Paul E Grundy, Conrad V Fernandez, Jeffrey S Dome
Purpose The National Wilms Tumor Study (NWTS) treatment of favorable histology Wilms tumor with lung metastases was vincristine/dactinomycin/doxorubicin (DD4A) and lung radiation therapy (RT). The AREN0533 study applied a new risk stratification and treatment strategy to improve event-free survival (EFS) while reducing exposure to lung RT. Methods Patients with favorable histology Wilms tumor and isolated lung metastases showing complete lung nodule response (CR) after 6 weeks of DD4A continued receiving chemotherapy without lung RT...
April 16, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29658966/methylation-in-the-promoter-regions-of-wt1-nkx6-1-and-dbc1-genes-in-cervical-cancer-tissues-of-uygur-women-in-xinjiang
#20
Dan Wu, Jinli Zhang, Peiwen Fan, Hongtao Li, Dongmei Li, Huan Pan, Hongchang He, Xianxian Ren, Zhenzhen Pan, Renfu Shao, Zemin Pan
This study aimed to explore: 1) DNA methylation in the promoter regions of Wilms tumor gene 1 (WT1), NK6 transcription factor related locus 1 gene (NKX6-1) and Deleted in bladder cancer 1 (DBC1) gene in cervical cancer tissues of Uygur women in Xinjiang, and 2) the correlation of gene methylation with the infection of HPV16/18 viruses. We detected HPV16/18 infection in 43 normal cervical tissues, 30 cervical intraepithelial neoplasia lesions (CIN) and 48 cervical cancer tissues with polymerase chain reaction (PCR) method...
January 2018: Genetics and Molecular Biology
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