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https://www.readbyqxmd.com/read/29351866/bioanalysis-of-a-panel-of-neurotransmitters-and-their-metabolites-in-plasma-samples-obtained-from-pediatric-patients-with-neuroblastoma-and-wilms-tumor
#1
Lucyna Konieczna, Anna Roszkowska, Teresa Stachowicz-Stencel, Anna Synakiewicz, Tomasz Bączek
This paper details the quantitative analysis of neurotransmitters, including dopamine (DA), norepinephrine (NE), epinephrine (E), and serotonin (5-HT), along with their respective precursors and metabolites in children with solid tumors: Wilms' tumor (WT) and neuroblastoma (NB). A panel of neurotransmitters was determined with the use of dispersive liquid-liquid microextraction (DLLME) technique combined with liquid-chromatography mass spectrometry (LC-MS/MS) in plasma samples obtained from a group of pediatric subjects with solid tumors and a control group of healthy children...
December 28, 2017: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/29343557/dicer1-and-associated-conditions-%C3%A2-identification-of-at-risk-individuals-and-recommended-surveillance-strategies
#2
Kris Ann P Schultz, Gretchen M Williams, Junne Kamihara, Douglas R Stewart, Anne K Harris, Andrew J Bauer, Joyce Turner, Rachana Shah, Katherine Schneider, Kami Wolfe Schneider, Ann Garrity Carr, Laura A Harney, Shari Baldinger, A Lindsay Frazier, Daniel Orbach, Dominik T Schneider, David Malkin, Louis P Dehner, Yoav H Messinger, Ashley Hill
Pathogenic germline DICER1 variants cause a hereditary cancer predisposition syndrome with a variety of manifestations. In addition to conferring increased cancer risks for pleuropulmonary blastoma (PPB) and ovarian sex cord-stromal tumors, particularly Sertoli-Leydig cell tumor, individuals with pathogenic germline DICER1 variants may also develop lung cysts, cystic nephroma, renal sarcoma and Wilms tumor, nodular hyperplasia of the thyroid, nasal chondromesenchymal hamartoma, ciliary body medulloepithelioma, genitourinary embryonal rhabdomyosarcoma and brain tumors including pineoblastoma and pituitary blastoma...
January 17, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29343077/delayed-onset-of-sleep-in-adolescents-with-pax6-haploinsufficiency
#3
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/29334535/simultaneous-presentation-of-wilms-tumor-and-contralateral-ganglioneuroma-in-a-child-case-report-and-literature-review
#4
Zuhal Bayramoglu, Ibrahim Adaletli, Emine Caliskan, Isin Kilicaslan, Cagla S Karaoglan, Alaattin Celik, Feryal Gun Soysal, Sema B Bay, Bulent Zulfikar
We demonstrate a 4-year-old girl who presented with progressive, asymmetrical, firm abdominal distention and was diagnosed with synchronous Wilms' tumor and left para-aortic ganglioneuroma (GN). Although synchronous tumors in the pediatric population are commonly associated with malignancy-predisposing syndromes, the patient in question was found to be otherwise healthy and had no clinical evidence nor family history of a syndrome. This case is the second one in the literature diagnosed with synchronous presentation of Wilms' tumor and GN in a previously healthy child...
January 12, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29333015/a-profile-of-pediatric-solid-tumors-a-single-institution-experience-in-kashmir
#5
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
#6
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/29320783/wt1-gene-mutation-p-r462w-in-a-46-xy-dsd-patient-from-egypt-with-gonadoblastoma-and-review-of-the-literature
#7
Inas Mazen, Heba Hassan, Alaa Kamel, Mona Mekkawy, Ken McElreavey, Mona Essawi
WT1 gene mutations have been described in 46,XY patients with ambiguous genitalia or complete gonadal dysgenesis with or without Wilms' tumor, nephropathy, gonadoblastoma, and other defects, e.g., cryptorchidism or hypospadias. p.R462W is a hot spot mutation in exon 9 and is the most common mutation in patients with Denys-Drash syndrome. However, in this study we report an Egyptian patient with a novel phenotype carrying the p.R462W mutation. We also review the heterogeneity of phenotypes of previously reported patients with the p...
January 11, 2018: Sexual Development: Genetics, Molecular Biology, Evolution, Endocrinology, Embryology, and Pathology of Sex Determination and Differentiation
https://www.readbyqxmd.com/read/29318365/-innovations-in-surgical-treatment-of-pediatric-solid-tumors
#8
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/29318103/cecal-volvulus-following-a-right-nephrectomy-for-wilms-tumor-should-we-need-to-close-the-lateral-peritoneum
#9
Mauricio Gonzalez-Urquijo, Christian Ovalle-Chao, Eduardo Flores-Villalba, Ulises de Jesus Garza-Luna, Jose Humberto Velazco-De La Garza, Ulises Garza-Serna
Wilms' tumor (WT) accounts for 90% of all pediatric renal malignant tumors. The most common postoperative complication based on the National Wilms' Tumor Study is small bowel obstruction. We report on a 2-year-old girl with postoperative bowel obstruction following a right nephrectomy for WT. The patient was reintervened 48 hours after surgery and a cecal volvulus was found. Here, we will describe possible causes of this postoperative complication and discuss management.
January 2018: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/29301605/extrarenal-wilms-tumor-of-the-female-genital-system-a-case-report-and-literature-review
#10
Min-Min Cao, Cui-Ping Huang, Ya-Fen Wang, De-Mei Ma
Extrarenal Wilms' Tumors (ERWTs) are rare. There have been only 25 cases of ERWT arising from the female genital system reported in the literature. In this paper, we report a 60-year-old woman with a complaint of vaginal bleeding and a polypoid mass in the uterine cavity by sonography that was demonstrated as ERWT by pathology after resection. The pathological characteristics, histological origination, diagnosis, therapy and prognosis of ERWT in female reproductive system are discussed in this paper in the purpose of improving the diagnosis and therapy of this rare tumor...
December 30, 2017: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/29300373/urine-cell-free-dna-is-a-biomarker-for-nephroblastomatosis-or-wilms-tumor-in-pik3ca-related-overgrowth-spectrum-pros
#11
Marta Biderman Waberski, Marjorie Lindhurst, Kim M Keppler-Noreuil, Julie C Sapp, Laura Baker, Karen W Gripp, Denise M Adams, Leslie G Biesecker
PurposeWe set out to facilitate the molecular diagnosis of patients with PIK3CA-related overgrowth spectrum (PROS), a heterogeneous somatic disorder characterized by variable presentations of segmental overgrowth, vascular malformations, skin lesions, and nephroblastomatosis, rare precursor lesions to Wilms tumor. Molecular diagnosis of PROS is challenging due to its mosaic nature, often requiring invasive biopsies.MethodsDigital droplet polymerase chain reaction (ddPCR) was used to analyze tissues including urine, saliva, buccal cells, and blood, from eight patients with PROS...
January 4, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
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
#12
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
#13
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
#14
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
#15
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
#16
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/29243987/association-between-hace1-gene-polymorphisms-and-wilms-tumor-risk-in-a-chinese-population
#17
Wei Jia, Zhijian Deng, Jinhong Zhu, Wen Fu, Shibo Zhu, Li-Yu Zhang, Jinhua Hu, Fenghua Wang, Huimin Xia, Guo-Chang Liu, Jing He
Wilms' tumor is one of the most common solid tumors of childhood; however, the genetic basis underlying the majority of cases remains largely unknown. HACE1 is a putative Wilms' tumor susceptibility gene. We investigated the association between five HACE1 gene polymorphisms and Wilms' tumor susceptibility in a Chinese population consisting of 145 patients and 531 controls. We found a significant association between HACE1 rs9404576 polymorphism and decreased Wilms' tumor risk. No significant association was detected for other polymorphisms in the overall analysis...
November 26, 2017: Cancer Investigation
https://www.readbyqxmd.com/read/29231959/causal-somatic-mutations-in-urine-dna-from-persons-with-the-cloves-subgroup-of-the-pik3ca-related-overgrowth-spectrum-pros
#18
M E Michel, D J Konczyk, K S Yeung, R Murillo, M P Vivero, A M Hall, D Zurakowski, D Adams, A Gupta, A Y Huang, B H Y Chung, M L Warman
Congenital Lipomatous Overgrowth with Vascular, Epidermal, and Skeletal anomalies (CLOVES) and Klippel-Trenaunay (KTS) syndromes are caused by somatic gain-of-function mutations in PIK3CA, encoding a catalytic subunit of phosphoinositide 3-kinase. Affected tissue is needed to find mutations, since mutant alleles are not detectable in blood. Because some patients with CLOVES develop Wilms tumor, we tested urine as a source of DNA for mutation detection. We extracted DNA from the urine of 17 and 24 individuals with CLOVES and KTS, respectively, and screened 5 common PIK3CA mutation hotspots using droplet digital PCR...
December 12, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/29230948/three-dimensional-printed-model-of-bilateral-wilms-tumor-a-useful-tool-for-planning-nephron-sparing-surgery
#19
Óscar Girón-Vallejo, Darío García-Calderón, Ramón Ruiz-Pruneda, Rosa Cabello-Laureano, Ernesto Doménech-Abellán, José Luis Fuster-Soler, José I Ruiz-Jiménez
Nephron sparing surgery (NSS) is increasingly utilized to treat patients with bilateral Wilms tumor. We present a case of NSS planning using a three-dimensional computerized and printed model of both kidneys with anatomical structures of interest (parenchyma, renal pelvis, major calyx, renal artery, renal vein, and tumor). This model allowed a better understanding of the anatomic relation between the tumor and the normal kidney on each side, improving the surgical planning and the preoperative discussion with the patient's family...
December 12, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29219666/coincidence-of-celiac-disease-with-nongastrointestinal-tumors-in-children
#20
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
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