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https://www.readbyqxmd.com/read/29343077/delayed-onset-of-sleep-in-adolescents-with-pax6-haploinsufficiency
#1
Alyson E Hanish, Joan C Han
OBJECTIVE: PAX6 haploinsufficiency ( +/-) can occur due to mutations involving only PAX6 in patients with isolated aniridia or as contiguous gene deletions in patients with Wilms tumor, aniridia, genitourinary anomalies, and range of developmental and intellectual disabilities syndrome. Given the role of PAX6 in pineal development and circadian regulation, adolescents with PAX6+/- may experience sleep-wake disturbances. The purpose of this observational study was to explore sleep-related phenotypes in adolescents with PAX6+/-...
January 1, 2018: Biological Research for Nursing
https://www.readbyqxmd.com/read/29333015/a-profile-of-pediatric-solid-tumors-a-single-institution-experience-in-kashmir
#2
Namita Sharma, Ayesha Ahmad, Gull M Bhat, Sheikh A Aziz, Mohammad Maqbool Lone, Nisar A Bhat
Aims: The purpose of this retroprospective study was to study the epidemiological characteristics and outcomes of children with solid tumors at our institution. Subjects and Methods: Three hundred and three pediatrics patients registered at Regional Cancer Centre (RCC), Sher-i-Kashmir Institute of Medical Sciences (SKIMS), Srinagar, Kashmir, between January 2008 and June 2014, were analyzed with regard to demographic status, presenting complaints, investigations, treatment, morbidity, and outcomes...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29333010/sinusoidal-obstruction-syndrome-during-treatment-for-wilms-tumor-a-life-threatening-complication
#3
Sidharth Totadri, Amita Trehan, Deepak Bansal, Richa Jain
Context: Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication. Aims: The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Settings and Design: Retrospective file review conducted in a Pediatric Hematology-Oncology unit...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29318365/-innovations-in-surgical-treatment-of-pediatric-solid-tumors
#4
REVIEW
A Schmidt, S W Warmann, C Urla, J Fuchs
The overall survival of children with solid tumors has shown a substantial increase in the past decades due to progress in all of the disciplines involved in the treatment. The poor prognosis for advanced stages of disease and the morbidity related to therapeutic procedures are still a challenge. Innovations in the surgical treatment of solid tumors can contribute to increase the survival rate of affected children and to decrease the treatment-related morbidity. Considering these aspects, the successful implementation of innovations is described based on four examples...
January 9, 2018: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
https://www.readbyqxmd.com/read/29285297/identification-of-wt1-as-determinant-of-heptatocellular-carcinoma-and-its-inhibition-by-chinese-herbal-medicine-salvia-chinensis-benth-and-its-active-ingredient-protocatechualdehyde
#5
Ning Wang, Hor-Yue Tan, Yau-Tuen Chan, Wei Guo, Sha Li, Yibin Feng
Candidates from Chinese herbal Medicine might be preferable in drug discovery as the abundant experiences of traditional use usually hint the clinical efficacy. In this study, we screened the anti-tumour effect of several commonly used Chinese herbal Medicines on human hepatocellular carcinoma cells (HCC). We identified that Salvia chinensia Benth. (Shijianchuan in Chinese, SJC) exhibited prominent in vitro inhibition of HCC cells and suppressed the orthotopic growth of HCC in the liver of mice and repressed the lung metastasis of tumour cells...
December 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/29274817/cytotoxicity-and-inhibition-of-leukemic-cell-proliferation-by-sesquiterpenes-from-rhizomes-of-mah-lueang-curcuma-cf-viridiflora-roxb
#6
Songyot Anuchapreeda, Nattakanwadee Khumpirapang, Kawinnat Rupitiwiriya, Leelawat Tho-Iam, Aroonchai Saiai, Siriporn Okonogi, Toyonobu Usuki
Curcuma cf. viridiflora Roxb., also known as Mah-Lueang in Thai, belongs to the Zingiberaceae family and is grown from rhizomes. The rhizome of the plant has been used for medicinal purposes, in particular, to treat paralysis in Thai traditional medicine. However, no biologically active compounds have been reported from Mah-Lueang yet. In this study, natural compounds were isolated from Mah-Lueang and structurally determined by spectroscopic methods, including electrospray ionization mass spectrometry and nuclear magnetic resonance...
December 13, 2017: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/29260287/change-in-liver-spleen-and-bone-marrow-magnetic-resonance-imaging-signal-intensity-over-time-in-children-with-solid-abdominal-tumors
#7
Michael Sirignano, Jonathan R Dillman, Brian D Weiss, Charles T Quinn, Bin Zhang, Weizhe Su, Andrew T Trout
BACKGROUND: Reticuloendothelial system MRI signal hypointensity is common in pediatric oncology patients with solid abdominal tumors. OBJECTIVE: To assess changes in liver, spleen and bone marrow T2-weighted MRI signal intensity over time and their relationship to blood transfusion history in children with solid abdominal tumors. MATERIALS AND METHODS: In this retrospective study we measured liver, spleen and bone marrow signal intensity on axial T2-weighted MR images obtained December 2009 through February 2016 in children with hepatoblastoma, neuroblastoma, ganglioneuroblastoma and Wilms tumor...
December 19, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/29247494/podocyte-and-endothelial-cell-injury-develop-nephrotic-syndrome-in-proliferative-lupus-nephritis
#8
Aya Nawata, Satoshi Hisano, Shohei Shimajiri, Ke-Yong Wang, Yoshiya Tanaka, Toshiyuki Nakayama
AIMS: Nephrotic syndrome (NS) is a major manifestation of lupus nephritis (LN). The dysregulation of podocyte, glomerular basement membrane (GBM) and endothelial cell (EC) develops proteinuria in glomerular diseases. The aim of our study is to clarify whether the dysregulation of these barriers is associated with NS in proliferative LN and membranous LN. METHODS AND RESULTS: Fifty six patients with NS including minimal change NS in 15, primary membranous nephropathy (PMN) in 13, Class III/IV LN in 15 and Class V LN in 13 were enrolled in this study...
December 15, 2017: Histopathology
https://www.readbyqxmd.com/read/29246796/current-state-of-renal-tumor-surgery-among-pediatric-surgeons-and-pediatric-urologists-a-survey-of-american-pediatric-surgical-association-apsa-and-society-for-pediatric-urology-spu-members
#9
Nicholas G Cost, Jennifer H Aldrink, Amanda F Saltzman, Roshni Dasgupta, Kenneth W Gow, Richard Glick, Peter F Ehrlich
INTRODUCTION: Anecdotally, renal tumor (RT) surgery makes up a limited portion of the practice for most pediatric urologists and pediatric surgeons. Data are lacking on the current perceptions of RT surgery, both volume of surgery and issues related to surgical practice, among pediatric surgeons (PS) and pediatric urologists (PU). OBJECTIVES: To describe practice patterns of pediatric renal tumor (RT) surgery and identify factors related to higher reported volumes of RT surgery...
December 2, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29219666/coincidence-of-celiac-disease-with-nongastrointestinal-tumors-in-children
#10
Majid Naderi, Iraj Shahramian, Mojtaba Delaramnasab, Ali Bazi
The association of celiac disease (CD) with cancers of gastrointestinal origin has been noted. However, coincidence of CD with nongastrointestinal neoplasms is an unusual event. Here we present five children with concurrent CD and nongastrointestinal neoplasms. All of the patients had positive serologic results for anti-tTG antibodies. Histological investigation of intestinal mucosa showed inflammation (Marsh score = 2) in all the patients. Two of these patients represented with germ cell malignancies. One patient had Wilms' tumor...
December 8, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29207642/crosstalk-in-competing-endogenous-rna-network-reveals-the-complex-molecular-mechanism-underlying-lung-cancer
#11
Xiang Jin, Yinghui Guan, Hui Sheng, Yang Liu
We investigated the transcriptional mechanism underlying lung cancer development. RNA sequencing analysis was performed on blood samples from lung cancer cases and healthy controls. Differentially expressed microRNAs (miRNAs), circular RNAs (circRNAs), mRNAs (genes), and long non-coding RNAs (lncRNA) were identified, followed by pathway enrichment analysis. Based on miRNA target interactions, a competing endogenous network was established and significant nodes were screened. Differentially expressed transcriptional factors were retrieved from the TRRUST database and the transcriptional factor regulatory network was constructed...
October 31, 2017: Oncotarget
https://www.readbyqxmd.com/read/29190181/identifying-opportunities-to-bridge-disparity-gaps-in-curing-childhood-cancer-in-malawi-malignancies-with-excellent-curative-potential-account-for-the-majority-of-diagnoses
#12
Nader Kim El-Mallawany, Peter Wasswa, Idah Mtete, Mercy Mutai, Christopher C Stanley, Mary Mtunda, Mary Chasela, Atupele Mpasa, Stella Wachepa, William Kamiyango, Jimmy Villiera, Peter N Kazembe, Parth S Mehta
The majority of African children with cancer die without access to resources. We describe efforts to build a public treatment program with curative intent for childhood cancer in Lilongwe, Malawi despite severe limitations in diagnostic and therapeutic resources. We retrospectively analyzed a cohort of childhood cancer patients at Kamuzu Central Hospital from 12/2011-6/2013. Consistently available chemotherapeutic agents were limited to cyclophosphamide, vincristine, doxorubicin, bleomycin, methotrexate, and prednisone...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29187024/ossifying-renal-tumor-of-infancy-report-of-a-case-with-positive-wt-1-immunohistochemistry-and-high-mitotic-index-and-review-of-the-literature
#13
Benoit Vaillancourt, Luc Oligny, Julie Déry, Julie Franc-Guimond, Dorothée Bouron-Dal Soglio
Ossifying renal tumor of infancy (ORTI) is a rare, benign pediatric tumor of the kidney. Since first reported by Chatten in 1980, 23 cases have been published. Previous authors have argued that ORTI might originate from nephrogenic rests, thereby sharing a pathogenic relationship with Wilms' tumor (WT). ORTI is characterized histologically by a population of polygonal osteoblast-like cells around an osteoid core and densely cellular component of blastemal-like or spindle cells. While the immunohistochemical profile of the cellular components has been reported, to the best of our knowledge, the status of WT1 expression has only been reported once, where it showed negative marking...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29170254/wt1-loss-attenuates-the-tp53-induced-dna-damage-response-in-t-cell-acute-lymphoblastic-leukemia
#14
Fulvio Bordin, Erich Piovan, Elena Masiero, Alberto Ambesi-Impiombato, Sonia Minuzzo, Roberta Bertorelle, Valeria Sacchetto, Giorgia Pilotto, Giuseppe Basso, Paola Zanovello, Alberto Amadori, Valeria Tosello
Loss of function mutations and deletions in Wilms tumor 1 (WT1) gene are present in about 10% of T-cell acute lymphoblastic leukemia. Clinically, (WT1) mutations are enriched in relapsed series and are associated to inferior relapse-free survival in thymic T-cell acute lymphoblastic leukemia cases. Here, we demonstrate that WT1 plays a critical role in DNA damage response in T-cell leukemia. (WT1) loss conferred resistance to DNA damaging agents and attenuated the transcriptional activation of important apoptotic regulators downstream of TP53 in TP53-competent MOLT4 T-leukemia cells but not in TP53-mutant T-cell acute lymphoblastic leukemia cell lines...
November 23, 2017: Haematologica
https://www.readbyqxmd.com/read/29165710/risk-of-soft-tissue-sarcoma-among-69-460-five-year-survivors-of-childhood-cancer-in-europe
#15
Chloe J Bright, Mike M Hawkins, David L Winter, Daniela Alessi, Rodrigue S Allodji, Francesca Bagnasco, Edit Bárdi, Andrea Bautz, Julianne Byrne, Elizabeth A M Feijen, Miranda M Fidler, Stanislaw Garwicz, Desiree Grabow, Thorgerdur Gudmundsdottir, Joyeeta Guha, Nadia Haddy, Momcilo Jankovic, Peter Kaatsch, Melanie Kaiser, Claudia E Kuehni, Helena Linge, Hilde Øfstaas, Cecile M Ronckers, Roderick Skinner, Jop C Teepen, Monica Terenziani, Giao Vu-Bezin, Finn Wesenberg, Thomas Wiebe, Carlotta Sacerdote, Zsuzsanna Jakab, Riccardo Haupt, Päivi Lähteenmäki, Lorna Zadravec Zaletel, Rahel Kuonen, Jeanette F Winther, Florent de Vathaire, Leontien C Kremer, Lars Hjorth, Raoul C Reulen
Background: Childhood cancer survivors are at risk of subsequent primary soft-tissue sarcomas (STS), but the risks of specific STS histological subtypes are unknown. We quantified the risk of STS histological subtypes after specific types of childhood cancer. Methods: We pooled data from 13 European cohorts, yielding a cohort of 69 460 five-year survivors of childhood cancer. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) were calculated. Results: Overall, 301 STS developed compared with 19 expected (SIR = 15...
November 20, 2017: Journal of the National Cancer Institute
https://www.readbyqxmd.com/read/29159937/diffusion-weighted-mri-and-intravoxel-incoherent-motion-model-for-diagnosis-of-pediatric-solid-abdominal-tumors
#16
Emma M Meeus, Niloufar Zarinabad, Karen A Manias, Jan Novak, Heather E L Rose, Hamid Dehghani, Katharine Foster, Bruce Morland, Andrew C Peet
BACKGROUND: Pediatric retroperitoneal tumors in the renal bed are often large and heterogeneous, and their diagnosis based on conventional imaging alone is not possible. More advanced imaging methods, such as diffusion-weighted (DW) MRI and the use of intravoxel incoherent motion (IVIM), have the potential to provide additional biomarkers that could facilitate their noninvasive diagnosis. PURPOSE: To assess the use of an IVIM model for diagnosis of childhood malignant abdominal tumors and discrimination of benign from malignant lesions...
November 21, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29154793/trends-in-urologic-indications-for-pediatric-renal-transplantation-over-a-27-year-period-unos-database
#17
Pankaj Dangle, Utsav Bansal, Rajeev Chaudhry, Glenn M Cannon, Francis X Schneck, Michael C Ost
OBJECTIVE: To elucidate the trends in urological causes for renal transplant in the pediatric population using a national database. Little is known about the specific pediatric urologic conditions and resultant trends that precede renal transplantation. MATERIAL AND METHODS: We reviewed the United Network for Organ Sharing (UNOS) database for pediatric patients (<18 years old) who underwent renal transplantation from January 1988- September 2015. We included those patients who received a renal transplant due to a urologic condition...
November 14, 2017: Urology
https://www.readbyqxmd.com/read/29152069/the-transcriptional-coregulator-nab2-is-a-target-gene-for-the-wilms-tumor-gene-1-protein-wt1-in-leukemic-cells
#18
Helena Jernmark Nilsson, Giorgia Montano, Tove Ullmark, Andreas Lennartsson, Kristina Drott, Linnea Järvstråt, Björn Nilsson, Karina Vidovic, Urban Gullberg
The Wilms' tumor gene 1 (WT1) is recurrently mutated in acute myeloid leukemia. Mutations and high expression of WT1 associate with a poor prognosis. In mice, WT1 cooperates with the RUNX1/RUNX1T1 (AML1/ETO) fusion gene in the induction of acute leukemia, further emphasizing a role for WT1 in leukemia development. Molecular mechanisms for WT1 are, however, incompletely understood. Here, we identify the transcriptional coregulator NAB2 as a target gene of WT1. Analysis of gene expression profiles of leukemic samples revealed a positive correlation between the expression of WT1 and NAB2, as well as a non-zero partial correlation...
October 20, 2017: Oncotarget
https://www.readbyqxmd.com/read/29138824/genetic-mutational-testing-of-chinese-children-with-familial-hematuria-with-biopsy%C3%A2-proven-fsgs
#19
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/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
#20
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
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