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https://www.readbyqxmd.com/read/28644979/intraoperative-fast-ripples-independently-predict-postsurgical-epilepsy-outcome-comparison-with-other-electrocorticographic-phenomena
#1
Shaun A Hussain, Gary W Mathern, Phoebe Hung, Julius Weng, Raman Sankar, Joyce Y Wu
In the surgical management of epilepsy, the resection of cortex exhibiting interictal fast ripples (250-500Hz) on electrocorticography has been linked to postoperative seizure-freedom. Although fast ripples appear to accurately identify the epileptogenic zone-the minimum tissue that must be removed at surgery to achieve seizure-freedom-it has not been established that fast ripples are a superior biomarker in comparison with multimodal presurgical neuroimaging and other electrocorticography abnormalities. Hence, in the prediction of postoperative seizure-freedom, we compared the value of fast ripples with other intraoperative electocorticography abnormalities including focal slowing, paroxysmal fast activity, intermittent spike discharges, continuous epileptiform discharges, focal attenuation, and intraoperative seizures, as well as complete resection of the lesion defined by MRI and other neuroimaging...
June 16, 2017: Epilepsy Research
https://www.readbyqxmd.com/read/28644838/characterization-and-utilization-of-an-international-neurofibromatosis-web-based-patient-entered-registry-an-observational-study
#2
Mindell Seidlin, Robert Holzman, Pamela Knight, Bruce Korf, Vanessa Rangel Miller, David Viskochil, Annette Bakker
The neurofibromatoses (neurofibromatosis type 1, neurofibromatosis type 2 and schwannomatosis) are rare disorders having clinical manifestations that vary greatly from patient to patient. The rarity and variability of these disorders has made it challenging for investigators to identify sufficient numbers of patients with particular clinical characteristics or specific germline mutations for participation in interventional studies. Similarly, because the natural history of all types of neurofibromatosis (NF) is variable and unique for each individual, it is difficult to identify meaningful clinical outcome measures for potential therapeutic interventions...
2017: PloS One
https://www.readbyqxmd.com/read/28644430/the-clinical-landscape-for-sma-in-a-new-therapeutic-era
#3
REVIEW
K Talbot, E F Tizzano
Despite significant advances in basic research, the treatment of degenerative diseases of the nervous system remains one of the greatest challenges for translational medicine. The childhood onset motor neuron disorder spinal muscular atrophy (SMA) has been viewed as one of the more tractable targets for molecular therapy, due to a detailed understanding of the molecular genetic basis of the disease. In SMA, inactivating mutations in the SMN1 gene can be partially compensated for by limited expression of SMN protein from a variable number of copies of the SMN2 gene, which provides both a molecular explanation for phenotypic severity and a target for therapy...
June 23, 2017: Gene Therapy
https://www.readbyqxmd.com/read/28644144/meta-analysis-of-the-clinical-characteristics-and-prognostic-relevance-of-notch1-and-fbxw7-mutation-in-t-cell-acute-lymphoblastic-leukemia
#4
Rong-Bin Liu, Jian-Gui Guo, Tian-Ze Liu, Cheng-Cheng Guo, Xin-Xiang Fan, Xiao Zhang, Xiu-Yu Cai, Wei-Han Hu
The NOTCH1 signaling pathway is crucial for T-cell development, and NOTCH1 and/or FBXW7 mutations are frequently detected in T-cell acute lymphoblastic leukemia (T-ALL). We performed a systematic review and meta-analysis of 18 randomized controlled trials (RCTs) to assess the prognostic impact of mutations in the NOTCH1 pathway. After retrieving relevant articles from PubMed, EMBASE, and the Cochrane Library, we investigated overall survival (OS) and event-free survival (EFS) with hazard ratios (HRs) using fixed-effects or random-effects models and conducted subgroup analyses based on population and mutation status...
June 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/28643931/clinical-effectiveness-of-late-maxillary-protraction-in-cleft-lip-and-palate-a-methods-paper
#5
M K Lee, C Lane, F Azeredo, M Landsberger, H Kapadia, B Sheller, S L Yen
OBJECTIVES: A prospective parallel cohort trial was conducted to compare outcomes of patients treated with maxillary protraction vs LeFort 1 maxillary advancement surgery. SETTING AND SAMPLE POPULATION: The primary site for the clinical trial is Children's Hospital Los Angeles; the satellite test site is Seattle Children's Hospital. All patients have isolated cleft lip and palate and a skeletal Class III malocclusion. MATERIAL AND METHODS: A total of 50 patients, ages 11-14, will be recruited for the maxillary protraction cohort...
June 2017: Orthodontics & Craniofacial Research
https://www.readbyqxmd.com/read/28642999/continually-improving-standards-of-care-the-uk-renal-registry-as-a-translational-public-health-tool
#6
REVIEW
Lucy A Plumb, Alexander J Hamilton, Carol D Inward, Yoav Ben-Shlomo, Fergus J Caskey
A disease registry uses observational study methods to collect defined data on patients with a particular condition for a predetermined purpose. By providing comprehensive standardised data on patients with kidney disease, renal registries aim to provide a 'real world' representation of practice patterns, treatment and patient outcomes that may not be captured accurately by other methods, including randomised controlled trials. Additionally, using registries to measure variations in outcomes and audit care against standards is crucial to understanding how to improve quality of care for patients in an efficacious and cost-effective manner...
June 22, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28642564/remineralisation-and-arresting-caries-in-children-with-topical-fluorides
#7
Neeraj Gugnani, Shalini Gugnani
Data sourcesThe Cochrane Library, PubMed, Embase and the ISI Web of Science.Study selectionEnglish language clinical trials in children with outcome measures including the remineralisation or arresting effect of caries by professional fluoride treatment were considered.Data extraction and synthesisTwo reviewers screened the studies and assessed risk of bias. Random effects meta-analysis was conducted.ResultsSeventeen studies were included, ten focused on remineralisation, seven on arresting carious lesions...
June 23, 2017: Evidence-based Dentistry
https://www.readbyqxmd.com/read/28642555/chlorhexidine-mouthwash-plaque-levels-and-gingival-health
#8
Derek Richards
Data sourcesCochrane Oral Health's Trials Register; Cochrane Central Register of Controlled Trials (CENTRAL) Medline; clinicaltrials.gov and the World Health Organization International Clinical Trials Registry Platform. There were no language or date restrictions on searches.Study selectionRandomised controlled trials (RCTs) assessing the effects of chlorhexidine mouthrinse used as an adjunct to mechanical oral hygiene procedures for at least four weeks on gingivitis in children and adults.Data extraction and synthesisTwo reviewers independently abstracted data and assessed risk of bias...
June 23, 2017: Evidence-based Dentistry
https://www.readbyqxmd.com/read/28642300/center-level-variation-in-accuracy-of-adverse-event-reporting-in-a-clinical-trial-for-pediatric-acute-myeloid-leukemia-a-report-from-the-children-s-oncology-group
#9
Tamara P Miller, Yimei Li, Marko Kavcic, Kelly D Getz, Yuan-Shun V Huang, Lillian Sung, Todd A Alonzo, Robert Gerbing, Marla H Daves, Terzah M Horton, Michael A Pulsipher, Jessica Pollard, Rochelle Bagatell, Alix E Seif, Brian T Fisher, Selina Luger, Alan S Gamis, Peter C Adamson, Richard Aplenc
No abstract text is available yet for this article.
June 22, 2017: Haematologica
https://www.readbyqxmd.com/read/28641756/regenerative-medicine-solutions-in-congenital-diaphragmatic-hernia
#10
Paolo De Coppi, Jan Deprest
Congenital diaphragmatic hernia (CDH) remains a major challenge and associated mortality is still significant. Patients have benefited from current therapeutic options, but most severe cases are still associated to poor outcome. Regenerative medicine is emerging as a valid option in many diseases and clinical trials are currently happening for various conditions in children and adults. We report here the advancement in the field which will help both in the understanding of further CDH development and in offering new treatment options for the difficult situations such as repair of large diaphragmatic defects and lung hypoplasia...
June 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/28641582/a-multicentre-randomised-controlled-trial-of-levetiracetam-versus-phenytoin-for-convulsive-status-epilepticus-in-children-protocol-convulsive-status-epilepticus-paediatric-trial-consept-a-predict-study
#11
Stuart R Dalziel, Jeremy Furyk, Megan Bonisch, Ed Oakley, Meredith Borland, Jocelyn Neutze, Susan Donath, Cynthia Sharpe, Simon Harvey, Andrew Davidson, Simon Craig, Natalie Phillips, Shane George, Arjun Rao, Nicholas Cheng, Michael Zhang, Kam Sinn, Amit Kochar, Christine Brabyn, Franz E Babl
BACKGROUND: Convulsive status epilepticus (CSE) is the most common life-threatening childhood neurological emergency. Despite this, there is a lack of high quality evidence supporting medication use after first line benzodiazepines, with current treatment protocols based solely on non-experimental evidence and expert opinion. The current standard of care, phenytoin, is only 60% effective, and associated with considerable adverse effects. A newer anti-convulsant, levetiracetam, can be given faster, is potentially more efficacious, with a more tolerable side effect profile...
June 22, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28641549/relevance-of-cat-and-dog-sensitization-by-skin-prick-testing-in-childhood-eczema-and-asthma
#12
Kam Lun E Lun Hon, Kathy Yin Ching Tsang, Ting Fan Leung
BACKGROUND: Household animal dander has been implicated as aeroallergen in childhood atopic diseases. Many parents seek healthcare advice if household pet keeping may be detrimental in atopic eczema (AE) and atopies. AIM: We investigated if skin sensitization by cat/dog dander was associated with disease severity and quality of life in children with AE. METHODS: Demographics, skin prick test (SPT) results, disease severity (Nottingham eczema severity score NESS), Children Dermatology Life Quality Index (CDLQI), blood IgE and eosinophil counts of a cohort of AE patients were reviewed...
June 14, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28640705/genomic-analysis-of-childhood-brain-tumors-methods-for-genome-wide-discovery-and-precision-medicine-become-mainstream
#13
Stephen C Mack, Paul A Northcott
Recent breakthroughs in next-generation sequencing technology and complementary genomic platforms have transformed our capacity to interrogate the molecular landscapes of human cancers, including childhood brain tumors. Numerous high-throughput genomic studies have been reported for the major histologic brain tumor entities diagnosed in children, including interrogations at the level of the genome, epigenome, and transcriptome, many of which have yielded essential new insights into disease biology. The nature of these discoveries has been largely platform dependent, exemplifying the usefulness of applying different genomic and computational strategies, or integrative approaches, to address specific biologic and/or clinical questions...
June 22, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28640697/current-clinical-challenges-in-childhood-ependymoma-a-focused-review
#14
Thomas E Merchant
Ependymoma is a locally aggressive tumor with metastatic potential that arises in diverse locations throughout the brain and spine in children. Tumor and treatment may result in significant morbidity. Cure remains elusive for many patients owing to diverse biology and resistance to conventional therapy. The implementation of systematic postoperative irradiation in clinical trials during the past 20 years has increased the proportion of patients achieving durable disease control with excellent results, as measured by objective functional outcome measures...
June 22, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28639564/effect-of-an-ecological-executive-skill-training-program-for-school-aged-children-with-attention-deficit-hyperactivity-disorder-a-randomized-controlled-clinical-trial
#15
Ying Qian, Min Chen, Lan Shuai, Qing-Jiu Cao, Li Yang, Yu-Feng Wang
BACKGROUND: As medication does not normalize outcomes of children with attention deficit hyperactivity disorder (ADHD), especially in real-life functioning, nonpharmacological methods are important to target this field. This randomized controlled clinical trial was designed to evaluate the effects of a comprehensive executive skill training program for school-aged children with ADHD in a relatively large sample. METHODS: The children (aged 6-12 years) with ADHD were randomized to the intervention or waitlist groups...
July 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28639398/epstein-barr-viral-loads-do-not-predict-post-transplant-lymphoproliferative-disorder-in-pediatric-lung-transplant-recipients-a-multicenter-prospective-cohort-study
#16
Andrew Parrish, Matthew Fenchel, Gregory A Storch, Richard Buller, Sheila Mason, Nikki Williams, David Ikle, Carol Conrad, Albert Faro, Samuel Goldfarb, Don Hayes, Ernestina Melicoff-Portillo, Marc Schecter, Gary Visner, Stuart Sweet, Lara Danziger-Isakov
Prediction of PTLD after pediatric lung transplant remains difficult. Use of EBV VL in WB has been poorly predictive, while measurement of VL in BAL fluid has been suggested to have enhanced utility. The NIH-sponsored Clinical Trials in Organ Transplantation in Children (CTOTC-03) prospectively obtained serial quantitative measurements of EBV PCR in both WB and BAL fluid after pediatric lung transplantation. Descriptive statistics, contingency analyses, and Kaplan-Meier analyses evaluated possible association between EBV and PTLD...
June 21, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28635579/-fish-oil-in-pregnancy-can-it-prevent-asthma-in-children
#17
P L P Brand, M B Katan
Taking fish oil supplements in the third trimester of pregnancy was associated with significantly less wheezing or asthma in the child at the age of 3-5 years, according to a randomized clinical trial by Bisgaard et al., NEJM 2017. However, the results of this study should be interpreted with caution. The primary end points were modified at a late stage in the study, and two primary end points, eczema in the first 3 years of life and allergic sensitization at 18 months of age, were demoted to secondary end points, and showed no significant effect of treatment...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28635157/longitudinal-study-on-the-use-of-dried-blood-spots-for-home-monitoring-in-children-after-kidney-transplantation
#18
A Al-Uzri, K A Freeman, Jordan Wade, K Clark, L A Bleyle, M Munar, D R Koop
The use of DBSs for home monitoring has been limited due to unsatisfactory blood sampling and analytical difficulties. The aim of this longitudinal feasibility trial was to assess the utility of DBS to monitor TAC and Cr at home in transplant recipients. A total of 30 participants (2-21 years, mean±SD, 13.6±5.4 year) were enrolled over 12 months. Eighteen were males. Monthly DBS samples were obtained at home and mailed to the central laboratory for analysis of TAC and Cr. Nineteen patients completed the study, and 216 cards were received in the laboratory from a total of 279 cards expected, with 416/519 (80%) blood spots being suitable for analysis...
June 20, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28634090/evaluation-of-the-effect-of-3-month-bladder-basic-advice-in-children-with-monosymptomatic-nocturnal-enuresis
#19
Marcin Tkaczyk, Michał Maternik, Anna Krakowska, Agnieszka Wosiak, Monika Miklaszewska, Katarzyna Zachwieja, Dariusz Runowski, Anna Jander, Dariusz Ratajczak, Agata Korzeniecka-Kozyrska, Izabella Mader-Wołyńska, Katarzyna Kiliś-Pstrusińska
INTRODUCTION: Advice (BBA) into the standards of patients' care in both monosymptomatic and non-monosymptomatic nocturnal enuresis. Although the idea of this recommendation was clear and reflects clinical experience, duration and efficacy have not been definitely established. Recent data have demonstrated the lack of efficacy of BBA and a fierce discussion has ensued. The present study was aimed to assess the efficacy of BBA in a group of previously untreated children with primary monosymptomatic nocturnal enuresis (MNE)...
June 1, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28633655/inhibition-of-mmp-2-and-mmp-9-decreases-cellular-migration-and-angiogenesis-in-in-vitro-models-of-retinoblastoma
#20
Anderson H Webb, Bradley T Gao, Zachary K Goldsmith, Andrew S Irvine, Nabil Saleh, Ryan P Lee, Justin B Lendermon, Rajini Bheemreddy, Qiuhua Zhang, Rachel C Brennan, Dianna Johnson, Jena J Steinle, Matthew W Wilson, Vanessa M Morales-Tirado
BACKGROUND: Retinoblastoma (Rb) is the most common primary intraocular tumor in children. Local treatment of the intraocular disease is usually effective if diagnosed early; however advanced Rb can metastasize through routes that involve invasion of the choroid, sclera and optic nerve or more broadly via the ocular vasculature. Metastatic Rb patients have very high mortality rates. While current therapy for Rb is directed toward blocking tumor cell division and tumor growth, there are no specific treatments targeted to block Rb metastasis...
June 20, 2017: BMC Cancer
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