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Grzegorz Jedrzejewski, Magdalena M Wozniak, Agata Pawelec, Albert Matera, Magdalena Kunach, Tomasz Madej, Andrzej P Wieczorek, Katarzyna Nowakowska
The aim of the ultrasound (US) screening program was to detect neoplastic lesions in children, together with other pathologies of the developmental age in the area of the neck, abdomen, female pelvis, and scrotum in boys.US screening scans, including cervical, abdominal, pelvical, and scrotal US, were performed in the population of asymptomatic children aged from 9 months to 6 years. The children were scanned in Mobile Pediatric US Unit, consisting of 2 independent consulting rooms.The scans of 14,324 children were analyzed, 7247 boys and 7077 girls...
October 2016: Medicine (Baltimore)
William J Gammerdinger, Matthew A Conte, Jean-François Baroiller, Helena D'Cotta, Thomas D Kocher
BACKGROUND: Inversions and other structural polymorphisms often reduce the rate of recombination between sex chromosomes, making it impossible to fine map sex-determination loci using traditional genetic mapping techniques. Here we compare distantly related species of tilapia that each segregate an XY system of sex-determination on linkage group 1. We use whole genome sequencing to identify shared sex-patterned polymorphisms, which are candidates for the ancestral sex-determination mutation...
October 18, 2016: BMC Genomics
Surbhi Goyal, Kiran Mishra, Urvee Sarkar, Satendra Sharma, Anita Kumari
BACKGROUND & OBJECTIVES: Renal tumours constitute about 7 per cent of all neoplasms in children. It is important to differentiate Wilms' tumour (commonest tumour) from non-Wilms' tumours. The aim of this study was to evaluate the immunoexpression and diagnostic role of Wilms' tumour-1 protein (WT1) in paediatric renal tumours. METHODS: A total of 53 cases of renal tumours in children (below 18 yr) who underwent total nephrectomy were included in this retrospective study...
May 2016: Indian Journal of Medical Research
Xiao-Su Zhao, Ya-Zhen Qin, Yan-Rong Liu, Ying-Jun Chang, Lan-Ping Xu, Xiao-Hui Zhang, Xiao-Jun Huang
The minimal residual disease (MRD) before and after a haploidentical hematopoietic stem cell transplantation (HSCT) of 86 patients with acute myeloid leukemia (AML) in complete remission (CR) was measured using flow cytometry (FCM) and Wilms tumor 1 (WT1). In all, 18 patients met the criteria of pre-MRD + before HSCT. The FCM + (p = .028) and the combinative criteria for positive MRD (MRDco+) (p = .022) post-transplantation were significantly correlated to relapse in univariate analysis. A multivariate analysis showed that only MRDco + post-transplantation was an independent risk factor of leukemia relapse (p = ...
October 12, 2016: Leukemia & Lymphoma
Mingsheng Zhang, Enda Xue, Wei Shao
BACKGROUND: Nephroblastoma (Wilms' tumor [WT]) is the most common malignant renal cancer in children. Although the outcome of WT has significantly improved as a result of the combination of surgery, chemotherapy, and radiotherapy; in some cases WT results in severe complications. Thus, novel strategies that would decrease treatment burden are required. The aim of the current study was to investigate the synergistic antitumor effect of andrographolide (AND) in combination with vincristine (VCR) on WT cells...
2016: Drug Design, Development and Therapy
Roberto Iglesias Lopes, Jessica Ming, Martin A Koyle, Ronald Grant, Adriana Fonseca, Armando J Lorenzo
OBJECTIVE: To describe the experience and technique of zero-ischemia laparoscopic-assisted partial nephrectomy at The Hospital for Sick Children, as an alternative to the traditional open approach for nephron sparing surgery in selected children with Wilms' tumors (WT). MATERIALS AND METHODS: Patients with diagnosis of WT treated with neoadjuvant chemotherapy and who underwent laparoscopic-assisted nephron-sparing surgery at the Hospital for Sick Children from 2012-2016 were identified and charts reviewed retrospectively...
October 6, 2016: Urology
Supamit Ukarapong, Gary Berkovitz, Kenneth McElreavey, Anu Bashamboo, Yong Bao
Mutation of the Wilms tumor suppressor gene (WT1) has been recognized as one of the etiologies of steroid-resistant nephrotic syndrome (SRNS). The mutation is also responsible for gonadal dysgenesis in 46,XY individuals. Early recognition of the presence of Y chromosome is of particular importance because of the high risk of gonadal tumor. We present here three cases of steroid-resistant nephrotic syndrome with WT1 mutation and 46,XY karyotype. Patient 1 and 2 have intron splice site (IVS9+5G > A) mutation...
October 10, 2016: Clinical Nephrology
Ariadne H A G Ooms, Samantha Gadd, Daniela S Gerhard, Malcolm A Smith, Jaime M Guidry Auvil, Daoud Meerzaman, Qing-Rong Chen, Chih Hao Hsu, Chunhua Yan, Cu Nguyen, Ying Hu, Yussanne Ma, Zusheng Zong, Andrew J Mungall, Richard A Moore, Marco A Marra, Vicki Huff, Jeffrey S Dome, Yueh-Yun Chi, Jing Tian, James I Geller, Charles G Mullighan, Jing Ma, David A Wheeler, Oliver A Hampton, Amy L Walz, Marry M van den Heuvel-Eibrink, Ronald R de Krijger, Nicole Ross, Julie M Gastier-Foster, Elizabeth J Perlman
PURPOSE: To investigate the role and significance of TP53 mutation in diffusely anaplastic Wilms tumors (DAWTs). EXPERIMENTAL DESIGN: All DAWTs registered on National Wilms Tumor Study-5 (n = 118) with available samples were analyzed for TP53 mutations and copy loss. Integrative genomic analysis was performed on 39 selected DAWTs. RESULTS: Following analysis of a single random sample, 57 DAWTs (48%) demonstrated TP53 mutations, 13 (11%) copy loss without mutation, and 48 (41%) lacked both [defined as TP53-wild-type (wt)]...
October 4, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Julia Hoefele, Markus J Kemper, Ulf Schoenermarck, Susanna Mueller, Hanns-Georg Klein, Anja Lemke
About 30% of children with nephrotic syndrome (NS) have inherited forms. Among them, mutations in Wilms tumor suppressor gene 1 (WT1) are a well characterized cause associated with steroid-resistant NS, Wilms tumor, and urogenital malformation in males. However, the role of WT1 mutations in adult-onset focal segmental glomerulosclerosis (FSGS) is unclear. We report the case of a 38-year-old female with FSGS. She had been diagnosed with streak ovaries during diagnostic workup for infertility. Mutational analysis identified the heterozygous mutation c...
October 5, 2016: Nephron
Korkiat Theerakitthanakul, Jeerasak Khrueathong, Jirasak Kruatong, Potchanapond Graidist, Pritsana Raungrut, Kanita Kayasut, Surasak Sangkhathat
: Wilms tumor (WT) is an embryonal tumor occurring in developing kidney tissue. WT cells showing invasive cancer characteristics, also retain renal stem cell behaviours. In-vitro culture of WT is hampered by limited replicative potential. This study aimed to establish a longterm culture of WT cells to enable the study of molecular events to attempt to explain its cellular senescence. METHODS: Primary cell cultures from fresh WT tumor specimen were established. Of 5 cultures tried, only 1 could be propagated for more than 7 passages...
2016: Journal of Cancer
Nadja Apelt, Jochen Hubertus, Doris Mayr, Norbert Graf, Rhoikos Furtwängler, Dietrich Von Schweinitz, Roland Kappler
Wilms tumor (WT) is the most common pediatric renal malignancy. A recent ontogenic model suggests that undifferentiated tumor state, and hence poor prognosis, in WT is determined by stabilization of β-catenin in the nucleus. Forkhead box M1 (FOXM1) is a downstream component of the Wnt pathway and promotes nuclear localization of β-catenin. As elevation of FOXM1 gene expression is prognostic in various types of malignancy, we hypothesized that high FOXM1 expression in WT is associated with undifferentiated histology and thus poor prognosis...
October 2016: Oncology Letters
Amei Schweigert, Carmen Fischer, Doris Mayr, Dietrich von Schweinitz, Roland Kappler, Jochen Hubertus
PURPOSE: The Wnt/β-catenin pathway is known to be crucial for the regulation of embryogenesis and cell differentiation, and its constitutive activation is associated with a wide range of malignancies. There are two major principles for an activated Wnt/β-catenin pathway. The first is caused by the failure of the destruction complex, mainly due to the decreased expression of the tumor suppressor gene adenomatous polyposis coli (APC); the second is the mutation of the β-catenin (CTNNB1) protein itself...
September 27, 2016: Pediatric Surgery International
J A Kalapurakal, M Gopalakrishnan, J W Jung, S Peterson, W Leisenring, F Laurie, C Rigsby, C Lee, T Fitzgerald, C Lee
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
T Morgan, H Danish, R H Nanda, L Meacham, N Esiashvili
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Z M Berhe, V Vanderpuye, Z Kassam, D Hodgson, J Yarney, N A Aryeetey, R Wong
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Mingming Xue, Yuru Zhou, Xiaoyu Liu, Dongsheng Ni, Yanxia Hu, Yaoshui Long, Pan Ju, Qin Zhou
To seek out the potential microRNAs (miRNAs) that target Wilms' tumor suppressor 1 (WT1), a transcription factor required for progenitor proliferation as well as normal development of the kidney, and to clarify the effects of the miRNAs on WT1, the 3'-untranslated region (3'‑UTR) of WT1 was initially analyzed and miR‑743a, a seldom‑reported miRNA, was identified. In the present paper, luciferase reporter assays were performed to confirm that miR‑743a is able to directly target the 3'‑UTR of WT1. Subsequently, reverse transcription‑quantitative polymerase chain reaction, combined with western blotting analyses, were performed, and the results revealed a significant inhibition of WT1 at the mRNA and the protein levels...
September 22, 2016: Molecular Medicine Reports
Ronell Bologna-Molina, Yasunori Takeda, Takahisa Kuga, Naoyuki Chosa, Masae Kitagawa, Takashi Takata, Akira Ishisaki, Toshinari Mikami
The Wilms' tumor 1 gene (WT1) was originally isolated and described as the gene responsible for Wilms' tumor. Although there is growing evidence linking the overexpression of WT1 to tumorigenesis, no reports on ameloblastoma are available at present. The aim of this study was to examine the expression of WT1 in various histological subtypes of ameloblastoma tissue specimens and in human ameloblastoma cell lines. Immunohistochemical analyses were performed on a total of 168 cases of ameloblastoma, one case of ameloblastic carcinoma, and five cases of tooth germs (control)...
2016: Journal of Oral Science
Yannick Willemen, Johan M J Van den Bergh, Sarah M Bonte, Sébastien Anguille, Carlo Heirman, Barbara M H Stein, Herman Goossens, Tessa Kerre, Kris Thielemans, Marc Peeters, Viggo F I Van Tendeloo, Evelien L J Smits, Zwi N Berneman
We formerly demonstrated that vaccination with Wilms' tumor 1 (WT1)-loaded autologous monocyte-derived dendritic cells (mo-DCs) can be a well-tolerated effective treatment in acute myeloid leukemia (AML) patients. Here, we investigated whether we could introduce the receptor for hyaluronic acid-mediated motility (RHAMM/HMMR/CD168), another clinically relevant tumor-associated antigen, into these mo-DCs through mRNA electroporation and elicit RHAMM-specific immune responses. While RHAMM mRNA electroporation significantly increased RHAMM protein expression by mo-DCs, our data indicate that classical mo-DCs already express and present RHAMM at sufficient levels to activate RHAMM-specific T cells, regardless of electroporation...
September 21, 2016: Oncotarget
Anissa Moktefi, Shao-Yu Zhang, Pauline Vachin, Virginie Ory, Carole Henique, Vincent Audard, Catherine Rucker-Martin, Elodie Gouadon, Michael Eccles, Andreas Schedl, Laurence Heidet, Mario Ollero, Djillali Sahali, Andre Pawlak
The WT1 (Wilm's tumor suppressor) gene is expressed throughout life in podocytes and is essential for the functional integrity of the glomerular filtration barrier. We have previously shown that CMIP (C-Maf inducing protein) is overproduced in podocyte diseases and alters intracellular signaling. Here we isolated the proximal region of the human CMIP promoter and showed by chromatin immunoprecipitation assays and electrophoretic-mobility shift that Wilm's tumor protein (WT1) bound to 2 WT1 response elements, located at positions -290/-274 and -57/-41 relative to transcription start site...
September 17, 2016: Kidney International
Nadine Bachmann, Roman Crazzolara, Florian Bohne, Dieter Kotzot, Kathrin Maurer, Thorsten Enklaar, Dirk Prawitt, Carsten Bergmann
BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is an early-onset overgrowth disorder with a high risk for embryonal tumors. It is mainly caused by dysregulation of imprinted genes on chromosome 11p15.5; however, the driving forces in the development of tumors are not fully understood. PROCEDURE: We report on a female patient presenting with macrosomia, macroglossia, organomegaly and extensive bilateral nephroblastomatosis. Adjuvant chemotherapy was initiated; however, the patient developed hepatoblastoma and Wilms tumor at 5 and 12 months of age, respectively...
September 21, 2016: Pediatric Blood & Cancer
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