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https://www.readbyqxmd.com/read/29329488/genetic-and-molecular-insights-into-genotype-phenotype-relationships-in-osteopathia-striata-with-cranial-sclerosis-oscs-through-the-analysis-of-novel-mouse-wtx-mutant-alleles
#1
Glenda Comai, Agnès Boutet, Kristina Tanneberger, Filippo Massa, Ana-Sofia Rocha, Aurelie Charlet, Clara Panzolini, Fariba Jian Motamedi, Robert Brommage, Wolfgang Hans, Thomas Funck-Brentano, Martin Hrabe de Angelis, Christine Hartmann, Martine Cohen-Solal, Jürgen Behrens, Andreas Schedl
The X-linked WTX/AMER1 protein forms an important component of the β-catenin destruction complex that can both enhance and suppress canonical β-catenin signalling. Somatic mutations in WTX/AMER1 have been found in a proportion of the pediatric kidney cancer Wilms' tumour. By contrast, germline mutations cause the severe sclerosing bone dysplasia osteopathia striata congenita with cranial sclerosis (OSCS), a condition usually associated with fetal or perinatal lethality in male patients. Here we addressed the developmental and molecular function of WTX by generating two novel mouse alleles...
January 12, 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/29329346/evaluation-of-bloodstream-infections-clostridium-difficile-infections-and-gut-microbiota-in-pediatric-oncology-patients
#2
Bryan T Nycz, Samuel R Dominguez, Deborah Friedman, Joanne M Hilden, Diana Ir, Charles E Robertson, Daniel N Frank
Bloodstream infections (BSI) and Clostridium difficile infections (CDI) in pediatric oncology/hematology/bone marrow transplant (BMT) populations are associated with significant morbidity and mortality. The objective of this study was to explore possible associations between altered microbiome composition and the occurrence of BSI and CDI in a cohort of pediatric oncology patients. Stool samples were collected from all patients admitted to the pediatric oncology floor from Oct.-Dec. 2012. Bacterial profiles from patient stools were determined by bacterial 16S rRNA gene profiling...
2018: PloS One
https://www.readbyqxmd.com/read/29328497/identification-of-theranostic-biomarkers-to-improve-the-stratification-of-patients-with-pediatric-liver-cancer-opportunities-and-challenges
#3
EDITORIAL
Carolina Armengol, Stefano Cairo
No abstract text is available yet for this article.
January 12, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29327612/the-evidence-for-expanded-genetic-testing-for-pediatric-patients-with-cancer
#4
Luke Maese, Joshua D Schiffman
No abstract text is available yet for this article.
January 12, 2018: Future Oncology
https://www.readbyqxmd.com/read/29326088/age-effects-of-distinct-immune-checkpoint-blockade-treatments-in-a-mouse-melanoma-model
#5
Álvaro Padrón, Vincent Hurez, Harshita B Gupta, Curtis A Clark, Sri Lakshmi Pandeswara, Bin Yuan, Robert S Svatek, Mary Jo Turk, Justin M Drerup, Rong Li, Tyler J Curiel
Cancer immunotherapy has shown remarkable recent progress. Immune checkpoint blocking antibodies have become the most successful anti-cancer agent class ever developed, with six distinct agents approved since 2011 for a wide variety of cancers. Although age is the biggest risk factor for cancer (aside from selected early-onset pediatric cancers), these agents were tested pre-clinically in young hosts, and there is remarkably little published on the effects of host age on treatment outcomes in pre-clinical studies or human clinical trials...
January 8, 2018: Experimental Gerontology
https://www.readbyqxmd.com/read/29325825/-thromboembolic-disease-in-pediatric-oncology
#6
A Theron, C Biron-Andreani, S Haouy, L Saumet, M Saguintah, E Jeziorski, N Sirvent
The survival rate of children with cancer is now close to 80 %, as a result of continuous improvement in diagnostic and treatment procedures. Prevention and treatment of treatment-associated complications is now a major challenge. Thromboembolic venous disease, due to multifactorial pathogenesis, is a frequent complication (up to 40 % asymptomatic thrombosis in children with cancer), responsible for significant morbidity. Predominantly in children with acute lymphoblastic leukemia, lymphoma, or sarcoma, thromboembolic disease justifies primary prophylaxis in certain populations at risk, whether genetic or environmental...
January 8, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29322843/immunotherapy-for-brain-tumors-understanding-early-successes-and-limitations
#7
Nicole A P Lieberman, Nicholas A Vitanza, Courtney A Crane
Adverse effects and toxicities related to standard treatments for brain tumors significantly reduce patients' quality of life. Although most immunotherapy approaches for solid tumors have not been successful, several early-phase clinical trials are beginning to reveal a potential role for immunotherapy in the treatment of brain tumors. In particular, methods that activate the innate immune system and induce a polyclonal anti-cancer response have demonstrated that brain tumors are susceptible to immune-mediated tumor destruction...
January 11, 2018: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/29322842/cd133-expression-and-mycn-amplification-induce-chemoresistance-and-reduce-average-survival-time-in-pediatric-neuroblastoma
#8
Zhi-Yong Zhong, Bao-Jun Shi, Hui Zhou, Wen-Bo Wang
Objectives Neuroblastoma (NB) is the most common pediatric solid tumor derived from the sympathetic nervous system. MYCN is amplified in nearly half of patients with NB, and its association with rapid disease progression and poor outcome is controversial. Characterization of cancer stem cells (CSCs) in NBs has been rarely studied. This study was performed to determine whether MYCN and CD133+ CSCs are associated with chemotherapy resistance and the survival time of patients with NB. Methods Fifty patients with an unequivocal pathological diagnosis of NB were recruited...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29319649/decreasing-radiation-exposure-in-pediatric-trauma-related-to-cervical-spine-clearance-a-quality-improvement-project
#9
Valerie Ann Waddell, Susan Connelly
STUDY DESIGN: Quality improvement project. OBJECTIVES: Reduce the amount of radiation exposure in the pediatric trauma population 5 years of age and older in relation to cervical spine clearance. BACKGROUND: The evaluation of pediatric cervical spine injuries must be accurate and timely to avoid missed injuries. The difficult clinical examination in pediatric trauma patients necessitates the use of radiologic examinations to avoid missing catastrophic injuries...
January 2018: Journal of Trauma Nursing: the Official Journal of the Society of Trauma Nurses
https://www.readbyqxmd.com/read/29319209/effectiveness-of-antibacterial-prophylaxis-during-induction-chemotherapy-in-children-with-acute-lymphoblastic-leukemia
#10
M L Sulis, T M Blonquist, K E Stevenson, S K Hunt, S Kay-Green, U H Athale, L A Clavell, P D Cole, K M Kelly, C Laverdiere, J M Leclerc, B Michon, M A Schorin, J G Welch, D S Neuberg, S E Sallan, L B Silverman
BACKGROUND: Pediatric patients receiving induction chemotherapy for newly diagnosed acute lymphoblastic leukemia (ALL) are at high risk of developing life-threatening infections. We investigated whether uniform antibacterial guidelines, including mandatory antibacterial prophylaxis in afebrile patients during induction, decreases the incidence of microbiologically documented bacteremia. METHODS: Between 2012 and 2015, 230 patients with newly diagnosed ALL (aged 1-21) were enrolled on Dana-Farber Cancer Institute ALL Consortium Protocol 11-001 (DFCI 11-001)...
January 10, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29317383/research-and-practice-based-nutrition-education-and-cooking-workshops-in-pediatric-oncology-protocol-for-implementation-and-development-of-curriculum
#11
Cynthia Chaput, Sabrina Beaulieu-Gagnon, Véronique Bélanger, Simon Drouin, Laurence Bertout, Lucie Lafrance, Cinthia Olivier, Marthe Robitaille, Caroline Laverdière, Daniel Sinnett, Marie Marquis, Valérie Marcil
BACKGROUND: Progresses in childhood cancer treatment, diagnosis, and management have resulted in childhood cancer survival rates of over 80%. However, this therapeutic success comes with a heavy price: two-thirds of childhood cancer survivors will be affected by further complications, including cardiovascular and metabolic diseases. Adequate nutrition during cancer treatment is essential to ensure the child's optimal development, improve tolerance to treatments, and can contribute to lower the risk of developing cardiometabolic diseases...
January 9, 2018: JMIR Research Protocols
https://www.readbyqxmd.com/read/29315962/multiple-dicer1-related-tumors-in-a-child-with-a-large-interstitial-14q32-deletion
#12
Leanne de Kock, Dominique Geoffrion, Barbara Rivera, Rabea Wagener, Nelly Sabbaghian, Susanne Bens, Benjamin Ellezam, Dorothée Bouron-Dal Soglio, Jessica Ordóñez, Stephanie Sacharow, Jose Fernando Polo Nieto, R Paul Guillerman, Gordan M Vujanic, John R Priest, Reiner Siebert, William D Foulkes
Germ-line interstitial deletions involving the 14q32 chromosomal region, resulting in 14q32 deletion syndrome, are rare. DICER1 is a recently described cancer-predisposition gene located at 14q32.13. We report the case of a male child with an approximately 5.8 Mbp 14q32.13q32.2 germ-line deletion, which included the full DICER1 locus. We reviewed available clinical and pathological material, and conducted genetic analyses. In addition to having congenital dysmorphic features, the child developed multiple DICER1 syndrome-related tumors before age 5 years: a pediatric cystic nephroma (pCN), a ciliary body medulloepithelioma (CBME), and a small lung cyst (consistent with occult pleuropulmonary blastoma Type I/Ir cysts seen in DICER1 mutation carriers)...
January 9, 2018: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/29315417/interactive-or-static-reports-to-guide-clinical-interpretation-of-cancer-genomics
#13
Stacy W Gray, Jeffrey Gagan, Ethan Cerami, Angel M Cronin, Hajime Uno, Nelly Oliver, Carol Lowenstein, Ruth Lederman, Anna Revette, Aaron Suarez, Charlotte Lee, Jordan Bryan, Lynette Sholl, Eliezer M Van Allen
Objective: Misinterpretation of complex genomic data presents a major challenge in the implementation of precision oncology. We sought to determine whether interactive genomic reports with embedded clinician education and optimized data visualization improved genomic data interpretation. Materials and Methods: We conducted a randomized, vignette-based survey study to determine whether exposure to interactive reports for a somatic gene panel, as compared to static reports, improves physicians' genomic comprehension and report-related satisfaction (overall scores calculated across 3 vignettes, range 0-18 and 1-4, respectively, higher score corresponding with improved endpoints)...
January 5, 2018: Journal of the American Medical Informatics Association: JAMIA
https://www.readbyqxmd.com/read/29315172/fertility-preservation-services-for-women-with-newly-diagnosed-cancer-a-national-assessment
#14
William D Winkelman, Mitchell P Rosen, Evelyn Mok-Lin
OBJECTIVE: To study the resources available for cancer patients once referred for fertility preservation. MATERIALS AND METHODS: Cross-sectional study clinics that are members of the Society for Assisted Reproductive Technology assess support for fertility preservation. RESULTS: Surveys were distributed to a total of 1135 members at 394 Society for Assisted Reproductive Technology clinics. Responses were received from 99 individuals representing a total of 84 unique clinics (21...
January 8, 2018: American Journal of Clinical Oncology
https://www.readbyqxmd.com/read/29315108/review-of-molecular-classification-and-treatment-implications-of-pediatric-brain-tumors
#15
Ana S Guerreiro Stucklin, Vijay Ramaswamy, Craig Daniels, Michael D Taylor
PURPOSE OF REVIEW: Brain tumors are the most common solid tumors and leading cause of cancer-related death in children. The advent of large-scale genomics has resulted in a plethora of profiling studies that have mapped the genetic and epigenetic landscapes of pediatric brain tumors, ringing in a new era of precision diagnostics and targeted therapies. In this review, we highlight the most recent findings, focusing on studies published after 2015, and discuss how new evidence is changing the care of children with brain tumors...
February 2018: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/29314654/exercise-program-for-children-and-adolescents-with-leukemia-and-lymphoma-during-treatment-a-comprehensive-review
#16
REVIEW
Giulia Zucchetti, Francesca Rossi, Carolina Chamorro Vina, Nicoletta Bertorello, Franca Fagioli
An exercise program (EP) during cancer treatment seems to be a valid strategy against physiological and quality-of-life impairments, but scientific evidence of benefits among pediatric patients is still limited. This review summarizes the literature focused on randomized controlled trials of EP offered to patients during leukemia and lymphoma treatment. Studies published up to June 2017 were selected from multiple databases and assessed by three independent reviewers for methodological validity. The review identified eight studies, but several types of bias have to be avoided to provide evidence-based recommendations accessible to patients, families, and professionals...
January 4, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29314142/retinoblastoma-the-visible-cns-tumor-a-review
#17
Helen Dimaras, Timothy W Corson
The pediatric ocular cancer retinoblastoma is the only central nervous system (CNS) tumor readily observed without specialized equipment: it can be seen by, and in, the naked eye. This accessibility enables unique imaging modalities. Here, we review this cancer for a neuroscience audience, highlighting these clinical and research imaging options, including fundus imaging, optical coherence tomography, ultrasound, and magnetic resonance imaging. We also discuss the subtype of retinoblastoma driven by the MYCN oncogene more commonly associated with neuroblastoma, and consider trilateral retinoblastoma, in which an intracranial tumor arises along with ocular tumors in patients with germline RB1 gene mutations...
January 3, 2018: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/29313943/pediatric-oncologist-willingness-to-offer-germline-tp53-testing-in-osteosarcoma
#18
Eliana Shaul, Michael Roth, Yungtai Lo, David S Geller, Bang Hoang, Rui Yang, David Malkin, Richard Gorlick, Jonathan Gill
BACKGROUND: Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome caused by mutations in the tumor-suppressor gene TP53. Osteosarcoma is a sentinel cancer in LFS. Prior studies using Sanger sequencing platforms have demonstrated that 3% of individuals with osteosarcoma harbor a mutation in TP53. New data from next-generation sequencing have demonstrated that 3.8% of patients with osteosarcoma have a known pathogenic variant, and an additional 5.7% carry exonic variants of unknown significance in TP53...
January 3, 2018: Cancer
https://www.readbyqxmd.com/read/29311160/development-of-chemotherapy-with-cell-cycle-inhibitors-for-adult-and-pediatric-cancer-therapy
#19
REVIEW
Christopher C Mills, E A Kolb, Valerie B Sampson
Preclinical and clinical development of agents that inhibit cell-cycle progression have brought an understanding of the feasibility of targeting various cell-cycle regulators in patients with cancer. Small molecule inhibitors targeting key proteins that participate in cell-cycle progression including the cyclin-dependent kinases and checkpoint kinases induce cell-cycle arrest and apoptosis in neoplastic cells. Early phase I studies demonstrate targeted inhibitors can be administered safely in adult and pediatric cancer patients, but these agents generally show limited clinical benefits as single agents...
January 8, 2018: Cancer Research
https://www.readbyqxmd.com/read/29309676/survival-impact-of-postoperative-radiotherapy-timing-in-pediatric-and-adolescent-medulloblastoma
#20
Alexander L Chin, Everett J Moding, Sarah S Donaldson, Iris C Gibbs, Scott G Soltys, Susan M Hiniker, Erqi L Pollom
Background: Radiation therapy (RT) remains a critical component of multimodality treatment for medulloblastoma. Traditionally, clinicians strive to start RT within 4-5 weeks of surgery, but the optimal timing after surgery remains unclear. Methods: Using the National Cancer Database, we identified pediatric and adolescent patients with medulloblastoma treated with curative-intent surgery, RT, and chemotherapy. Factors associated with early or delayed RT were identified using Pearson chi-squared tests...
January 4, 2018: Neuro-oncology
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