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Paediatric malignancy

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https://www.readbyqxmd.com/read/28650469/a-compartmentalized-phosphoinositide-signaling-axis-at-cilia-is-regulated-by-inpp5e-to-maintain-cilia-and-promote-sonic-hedgehog-medulloblastoma
#1
S E Conduit, V Ramaswamy, M Remke, D N Watkins, B J Wainwright, M D Taylor, C A Mitchell, J M Dyson
Sonic Hedgehog (SHH) signaling at primary cilia drives the proliferation and progression of a subset of medulloblastomas, the most common malignant paediatric brain tumor. Severe side effects associated with conventional treatments and resistance to targeted therapies has led to the need for new strategies. SHH signaling is dependent on primary cilia for signal transduction suggesting the potential for cilia destabilizing mechanisms as a therapeutic target. INPP5E is an inositol polyphosphate 5-phosphatase that hydrolyses PtdIns(4,5)P2 and more potently, the phosphoinositide (PI) 3-kinase product PtdIns(3,4,5)P3...
June 26, 2017: Oncogene
https://www.readbyqxmd.com/read/28641192/obstructive-neonatal-atrial-myxoma
#2
Federica Iezzi, Andrea Quarti, Alessandro Capestro, Marco Pozzi
INTRODUCTION: Cardiac myxoma is a benign neoplasm representing the most common primary cardiac tumor in adults, however it is unusual in neonates. It is represented by an endocardial mass that occupies the cardiac chamber. Although the majority of myxomas are attached to the fossa ovalis of the interatrial septum, they also attach to the walls of the cardiac chambers and to valve leaflets surfaces. Approximately 75% of myxomas are found in the left atrium, 20% are located in the right atrium, and rarely in the ventricles...
June 8, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28627156/-tnm-classification-of-malignant-tumours-eighth-edition-which-news
#3
Silvia Patriarca, Stefano Ferretti, Roberto Zanetti
The eighth edition of TNM classification of malignant tumours has been published by the Union for International Cancer Control in January 2017. As for the previous editions - from the third on - it has been translated into Italian and recently published in our Country. This article explains the main changes from the previous edition: new classifications, some major revisions of cancer staging rules, the introduction of a grid of prognostic factors for each neoplasia and the addition of two chapters. These two chapters are about the essential TNM and the paediatric tumours, and have been developed in order to facilitate the use of data by Cancer Registries...
January 2017: Epidemiologia e Prevenzione
https://www.readbyqxmd.com/read/28623181/allele-level-hla-matching-for-umbilical-cord-blood-transplantation-for-non-malignant-diseases-in-children-a-retrospective-analysis
#4
Mary Eapen, Tao Wang, Paul A Veys, Jaap J Boelens, Andrew St Martin, Stephen Spellman, Carmem Sales Bonfim, Colleen Brady, Andrew J Cant, Jean-Hugues Dalle, Stella M Davies, John Freeman, Katherine C Hsu, Katharina Fleischhauer, Chantal Kenzey, Joanne Kurtzberg, Gerard Michel, Paul J Orchard, Annalisa Paviglianiti, Vanderson Rocha, Michael R Veneris, Fernanda Volt, Robert Wynn, Stephanie J Lee, Mary M Horowitz, Eliane Gluckman, Annalisa Ruggeri
BACKGROUND: The standard for selecting unrelated umbilical cord blood units for transplantation for non-malignant diseases relies on antigen-level (lower resolution) HLA typing for HLA-A and HLA-B, and allele-level for HLA-DRB1. We aimed to study the effects of allele-level matching at a higher resolution-HLA-A, HLA-B, HLA-C, and HLA-DRB1, which is the standard used for adult unrelated volunteer donor transplantation for non-malignant diseases-for umbilical cord blood transplantation...
June 13, 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28618012/metabolomic-signature-of-brain-cancer
#5
Renu Pandey, Laura Caflisch, Alessia Lodi, Andrew J Brenner, Stefano Tiziani
Despite advances in surgery and adjuvant therapy, brain tumours represent one of the leading causes of cancer-related mortality and morbidity in both adults and children. Gliomas constitute about 60% of all cerebral tumours, showing varying degrees of malignancy. They are difficult to treat due to dismal prognosis and limited therapeutics. Metabolomics is the untargeted and targeted analyses of endogenous and exogenous small molecules, which characterizes the phenotype of an individual. This emerging "omics" science provides functional readouts of cellular activity that contribute greatly to the understanding of cancer biology including brain tumour biology...
June 15, 2017: Molecular Carcinogenesis
https://www.readbyqxmd.com/read/28615821/pyogenic-granuloma-like-lesion-in-children-should-we-wait
#6
Lai Kuan Chooi, Arman Zaharil Mat Saad, Shamala Durairajanayagam
Malignant melanoma is a potentially lethal cutaneous malignancy. Melanoma in paediatrics is rare as compared to adult melanoma. The clinicopathological characteristics of paediatric melanoma are different from adult melanoma, and the presence of melanoma mimics which occurs frequently in children (Spitz naevi) resulted in diagnosis uncertainty. We reported a 9-year-old girl who presented with a slow-growing, pyogenic granuloma-like lesion which was diagnosed with melanoma. It is important to have a high index of suspicion in paediatric skin lesion that would usually be deemed benign...
January 2017: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
https://www.readbyqxmd.com/read/28604107/accelerating-drug-development-for-neuroblastoma-new-drug-development-strategy-an-innovative-therapies-for-children-with-cancer-european-network-for-cancer-research-in-children-and-adolescents-and-international-society-of-paediatric-oncology-europe-neuroblastoma
#7
Lucas Moreno, Hubert Caron, Birgit Geoerger, Angelika Eggert, Gudrun Schleiermacher, Penelope Brock, Dominique Valteau-Couanet, Louis Chesler, Johannes H Schulte, Katleen De Preter, Jan Molenaar, Alexander Schramm, Martin Eilers, Tom Van Maerken, John Inge Johnsen, Michelle Garrett, Sally L George, Deborah A Tweddle, Per Kogner, Frank Berthold, Jan Koster, Giuseppe Barone, Elizabeth R Tucker, Lynley Marshall, Ralf Herold, Jaroslav Sterba, Koen Norga, Gilles Vassal, Andrew Dj Pearson
Neuroblastoma, the commonest paediatric extra-cranial tumour, remains a leading cause of death from cancer in children. There is an urgent need to develop new drugs to improve cure rates and reduce long-term toxicity and to incorporate molecularly targeted therapies into treatment. Many potential drugs are becoming available, but have to be prioritised for clinical trials due to the relatively small numbers of patients. Areas covered: The current drug development model has been slow, associated with significant attrition, and few new drugs have been developed for neuroblastoma...
June 26, 2017: Expert Opinion on Drug Discovery
https://www.readbyqxmd.com/read/28588177/antibiotic-susceptibility-pattern-of-bacteria-isolated-from-various-specimens-of-patients-with-hematological-malignancy
#8
J Roy, D C Nag
Infection is a life threatening complication in patients with hematological malignancy. So, proper treatment of infection with suitable antibiotic is very important in these patients. The aim of this study was to determine the antibiotic susceptibility of bacteria isolated from various specimens of patients with hematological malignancy, so that, an appropriate regimen of empiric antibiotic treatment can be established for these patients. This observational study was done in the Department of Microbiology and Immunology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from March 2012 to August 2012...
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28587626/evaluation-of-a-novel-antibody-to-define-histone-3-3-g34r-mutant-brain-tumours
#9
Farhana Haque, Pascale Varlet, Julien Puntonet, Lisa Storer, Aikaterini Bountali, Ruman Rahman, Jacques Grill, Angel M Carcaboso, Chris Jones, Robert Layfield, Richard G Grundy
Missense somatic mutations affecting histone H3.1 and H3.3 proteins are now accepted as the hallmark of paediatric diffuse intrinsic pontine gliomas (DIPG), non-brain stem paediatric high grade gliomas (pHGG) as well as a subset of adult glioblastoma multiforme (GBM). Different mutations give rise to one of three amino acid substitutions at two critical positions within the histone tails, K27M, G34R/V. Several studies have highlighted gene expression and epigenetic changes associated with histone H3 mutations; however their precise roles in tumourigenesis remain incompletely understood...
June 6, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28586748/haematological-malignancies-following-temozolomide-treatment-for-paediatric-high-grade-glioma
#10
Michael Karremann, Nadja Krämer, Marion Hoffmann, Maria Wiese, Andreas Beilken, Selim Corbacioglu, Dagmar Dilloo, Pablo Hernáiz Driever, Wolfram Scheurlen, Andreas Kulozik, Gerrit H Gielen, André O von Bueren, Matthias Dürken, Christof M Kramm
BACKGROUND: Temozolomide (TMZ) is widely used in high-grade glioma (HGG). There is a major concern of treatment-induced secondary haematological malignancies (SHMs). Due to the poor overall survival of HGG patients, the true incidence is yet elusive. Thus, the aim of this study was to determine the risk of SHMs following TMZ in paediatric HGG. METHODS: We analysed 487 patients from the HIT-HGG database of the German-speaking Society of Pediatric Oncology and Hematology with follow up beyond 1 year...
June 3, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28571287/surgical-management-of-a-rare-case-of-massive-compound-odontome-associated-with-missing-primary-tooth
#11
Elengkumaran Sankaran, Sangeetha Narasimhan, K S Sabitha, Punit Chitlangia
Odontomes are considered to be the most common odontogenic tumours of the jaws. They are benign, mixed tumours arising from the remnants of both odontogenic epithelium and the ectomesenchyme resulting in the deposition of varied propotions of enamel, dentin, cementum and pulp tissues. As these lesions show deficiency only in structural arrangement, some authors consider odontomes as hamartomas or tumour like malformations. Though these lesions are more common in children, very few cases have been reported in less than five years age group...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28524223/-infantile-epileptic-encephalopathies-what-matters-is-genetics
#12
J J Garcia-Penas, M Jimenez-Legido
INTRODUCTION: Epileptic encephalopathies in infancy are defined as conditions where the sustained epileptic activity itself may contribute to the severe neurological and cognitive impairment. These epileptic encephalopathies include Ohtahara syndrome, early myoclonic epileptic encephalopathy, West syndrome, Dravet syndrome, and malignant migrating epilepsy in infancy. These syndromes result from identifiable primary causes, such as structural, neurodegenerative, metabolic, or genetic defects...
May 17, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28524064/longitudinal-melanonychia-in-childhood-a-clinical-and-histopathological-review-of-korean-patients
#13
Min Kyung Lee, Suk Bae Seo, Ji Yun Jung, Yo Sup Shin, Eun Byul Cho, Eun Joo Park, Kwang Ho Kim, Kwang Joong Kim
Longitudinal melanonychia is not frequently observed in children, and few studies exist on longitudinal melanonychia in paediatric patients, especially in Asian populations. We aimed to evaluate the clinical and histological findings of longitudinal melanonychia in Korean paediatric patients. A retrospective review of paediatric patients (≤18 years old) with longitudinal melanonychia who underwent nail biopsy between January 2010 and October 2015 was performed. Of a total of 21 patients with longitudinal melanonychia, there were 10 cases of functional melanonychia, eight of lentigo, and three of nevus, as determined by microscopic examination...
May 19, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28516188/management-of-bilateral-wilms-tumours
#14
REVIEW
Alastair J W Millar, Sharon Cox, Alan Davidson
Wilms tumour is named after Max Wilms. It is an embryonal tumour derived from the metanephros. It is the commonest childhood renal tumour and the third commonest paediatric malignancy. Synchronous bilateral Wilms tumours (BWT) represent 4-7% of all Wilms tumours (WT) and present at a younger age than unilateral Wilms tumours. At least 10% of synchronous BWTs have unfavourable histology, and up to 22% are associated with genitourinary abnormalities, aniridia, WAGR syndrome, Denys-Drash syndrome, hemihypertrophy, or one of the other overgrowth syndromes...
May 17, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28511396/a-clinicopathological-study-of-paediatric-liver-tumours-in-a-tertiary-care-hospital
#15
Esakki Muthuvel, Vimal Chander, Chitra Srinivasan
INTRODUCTION: Paediatric primary liver tumours are the third largest group of solid abdominal neoplasms in children next to neuroblastoma and Wilms tumour, accounting for about 0.5% to 2% of all paediatric neoplasms, in which hepatoblastoma is the most common. AIM: The present study was done to estimate the incidence of paediatric liver tumours over a period of five years and also, to study the clinical behaviour, alpha-fetoprotein correlation and histopathological features of paediatric liver tumours...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28508875/early-phase-clinical-trials-of-anticancer-agents-in-children-and-adolescents-an-itcc-perspective
#16
REVIEW
Lucas Moreno, Andrew D J Pearson, Xavier Paoletti, Irene Jimenez, Birgit Geoerger, Pamela R Kearns, C Michel Zwaan, Francois Doz, Andre Baruchel, Josef Vormoor, Michela Casanova, Stefan M Pfister, Bruce Morland, Gilles Vassal
In the past decade, the landscape of drug development in oncology has evolved dramatically; however, this paradigm shift remains to be adopted in early phase clinical trial designs for studies of molecularly targeted agents and immunotherapeutic agents in paediatric malignancies. In drug development, prioritization of drugs on the basis of knowledge of tumour biology, molecular 'drivers' of disease and a drug's mechanism of action, and therapeutic unmet needs are key elements; these aspects are relevant to early phase paediatric trials, in which molecular profiling is strongly encouraged...
May 16, 2017: Nature Reviews. Clinical Oncology
https://www.readbyqxmd.com/read/28508462/role-of-diffusion-weighted-imaging-in-distinguishing-thoracoabdominal-neuroblastic-tumours-of-various-histological-types-and-differentiation-grades
#17
Yang Wen, Yun Peng, Xiao Min Duan, Nan Zhang
INTRODUCTION: Diffusion-weighted MR imaging (DWI) has demonstrated a great potential to help distinguish benign from malignant tumours in paediatric body. Our purpose is to evaluate whether DWI allow discrimination of thoracoabdominal neuroblastic tumours of various histological types and differentiation grades. METHODS: We retrospectively analysed DWI scans of the thoracoabdominal neuroblastic tumours in 25 children (11 girls, 14 boys). DWI was performed with two b-values of 0 and 800 s/mm(2) on a 3...
May 16, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28504866/a-short-report-on-current-fertility-preservation-strategies-for-boys
#18
Wei Li Cindy Ho, Harold Bourne, Debra Gook, Gary Clarke, Matthew Kemertzis, Kate Stern, Franca Agresta, Yves Heloury, Hannah Clarke, Lisa Orme, Michael Sullivan, Yasmin Jayasinghe, Margaret R Zacharin
BACKGROUND: Advances in cancer treatment have led to improved long term survival after childhood cancer, but often at a price of impaired future fertility. Fertility Preservation (FP) in male children and early adolescents poses unique challenges as efficacy is unproven. OBJECTIVES: To describe characteristics of testicular tissue cryopreservation (TTCP) specimens taken from paediatric and adolescent patients, stratified by age, and prior chemotherapy, if any, and to demonstrate evidence for germ cells MATERIALS AND METHODS: Retrospective review of gonadal biopsies and clinical records of patients consented into the Royal Children's Hospital FP program between 1987-2015...
May 15, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28501946/application-of-international-caries-detection-and-assessment-system-icdas-and-caries-management-by-risk-assessment-cambra-systems-in-child-cancer-patients-a-clinical-case-report
#19
A Hernández Fernández, R E Oñate Sánchez, E Fernández Miñano, P Iniesta López-Matencio, A J Ortiz Ruiz
BACKGROUND: Leukaemia represents 30-40% of all paediatric malignant tumours and is the main cause of death in patients aged <15 years. One of the main complications in these patients is infection, which may often occur in the oral cavity. Chemotherapy-related oral health problems may be reduced by oral healthcare strategies based on the International Caries Detection and Assessment System (ICDAS) and Caries Management by Risk Assessment (CABRA). CASE REPORT: A case is reported of a 14-year-old girl treated for leukaemia who presented with established dental caries lesions which were classified and treated according to ICDAS and CABRA protocols...
May 13, 2017: European Archives of Paediatric Dentistry: Official Journal of the European Academy of Paediatric Dentistry
https://www.readbyqxmd.com/read/28499444/a-french-national-breast-and-thyroid-cancer-screening-programme-for-survivors-of-childhood-adolescent-and-young-adult-caya-cancers-denacapst-programme
#20
Charlotte Demoor-Goldschmidt, Delphine Drui, Isabelle Doutriaux, Gérard Michel, Pascal Auquier, Agnès Dumas, Claire Berger, Valérie Bernier, Sandrine Bohrer, Pierre-Yves Bondiau, Bruno Filhon, Brice Fresneau, Claire Freycon, Dinu Stefan, Sylvie Helfre, Angela Jackson, Christine Kerr, Anne Laprie, Julie Leseur, Marc-André Mahé, Caroline Oudot, Claire Pluchard, Stéphanie Proust, Hélène Sudour-Bonnange, Céline Vigneron, Nathalie Lassau, Martin Schlumberger, Cécile Faure Conter, Florent de Vathaire
BACKGROUND: Survival of childhood, adolescent and young adult (CAYA) cancers has increased with progress in the management of the treatments and has reached more than 80% at 5 years. Nevertheless, these survivors are at great risk of second cancers and non-malignant co-morbidities in later life. DeNaCaPST is a non-interventional study whose aim is to organize a national screening for thyroid cancer and breast cancer in survivors of CAYA cancers. It will study the compliance with international recommendations, with the aim, regarding a breast screening programme, of offering for every woman living in France, at equal risk, an equal screening...
May 12, 2017: BMC Cancer
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