Hit the target: language activities designed to develop simple grammatical structures

This paper discusses language therapy activities for helping hearing impaired children acquire language

Safety and efficacy of alternative alglucosidase alfa regimens in Pompe disease

AbstractEmerging phenotypes in long-term survivors with Pompe disease on standard enzyme replacement therapy (ERT) (alglucosidase alfa 20 mg/kg/2 weeks) can include patients with worsening motor function. Whether higher doses of ERT improve skeletal function in these patients has not been systematically studied. This exploratory, randomized, open-label, 52-week study examined the safety and efficacy of 2 ERT regimens of alglucosidase alfa (20 mg/kg/week or 40 mg/kg/2 weeks) in 13 patients with Pompe disease and clinical decline or a lack of improvement on standard ERT: late-onset (n = 4), infantile-onset (n = 9). Cross-reactive immunologic material assay-negative patients were excluded. Eleven of 13 patients completed the study. Trends for improvement were seen in total gross motor function, but not mobility; however, 6 (late-onset, 2; infantile-onset, 4) of 11 patients (55%) who met the entry criteria of motor decline (late-onset, 4; infantile-onset, 7) showed improvement in motor and/or mobility skills. No between-regimen differences in efficacy emerged. Two case studies highlight the benefits of increased ERT dose in patients with Pompe disease experiencing clinical decline. Both alternative regimens were generally well tolerated. This study was limited by the small sample size, which is not uncommon for small clinical studies of rare diseases. Additionally, the study did not include direct assessment of muscle pathology, which may have identified potential causes of decreased response to ERT. Results were inconclusive but suggest that increased ERT dose may be beneficial in some patients with Pompe disease experiencing motor decline. Controlled studies are needed to clarify the benefits and risks of this strategy

The Grizzly, October 22, 1982

Ferry Named Ursinus Queen • Unique Course Offered • U.C. Choir Presents Bach • Night School Enrollment Up • 1983 Spring Registration • Letters to the Editor • President\u27s Corner • Lewis on Wall Street • Half a Great Show • Watching the Boob Tube • Homecoming \u2782 • And to Prove My Point • New Bus Schedule • Washington Semester: Get Out of Here • Bear Pack Falls to Stiff Competition • Soccer Conquers Albright and Alumni • Grapplers Take to the Mats • Grizzlies Suffer Homecoming Setback • Delaware tops Lady Bearshttps://digitalcommons.ursinus.edu/grizzlynews/1085/thumbnail.jp

Expressions 1985

PRODUCTION STAFFLOUISE AUSTINDANA Z. BEHRENDSENAMBER HEWITTTOSHIA SOUTHRANDY STANDLEYSHELLY WILSONhttps://openspace.dmacc.edu/expressions/1006/thumbnail.jp

Fortifying or fragmenting the state? The political economy of the drug trade in Shan State, Myanmar, 1988-2012

Over the past twenty-five years, the government of Myanmar (Burma) has consolidated control over large parts of Shan State, neutralizing much of the threat posed by armed groups and strengthening its hold over revenue extraction. During this period Myanmar has retained its position as the world's second largest producer of illicit opium, much of which is converted into heroin within the country's borders. This article explores the relationship between state-building processes and the illicit opium/heroin economy in Shan State since 1988. The author has four aims. First, to reassess the theoretical assumptions that equate illicit economies with state fragility and demonstrate instead why illicit drug economies can become embedded in processes of conflict reduction and state consolidation. Second, to explain why establishing control over Shan State has become so important to the Myanmar government's state-building ambitions. Third, to analyze how the state's engagement with the drug trade has become a vital part of its attempts to consolidate control, in terms of financing military expansion and brokering deals with strongmen who are able to govern local populations. Finally, to assess how these strategies embody a form of “negotiated statehood” in which the state's growing control has been defined by attempts to manage, rather than monopolize, the means of coercion and extraction

Development and Validation of the Gene Expression Predictor of High-grade Serous Ovarian Carcinoma Molecular SubTYPE (PrOTYPE).

PURPOSE: Gene expression-based molecular subtypes of high-grade serous tubo-ovarian cancer (HGSOC), demonstrated across multiple studies, may provide improved stratification for molecularly targeted trials. However, evaluation of clinical utility has been hindered by nonstandardized methods, which are not applicable in a clinical setting. We sought to generate a clinical grade minimal gene set assay for classification of individual tumor specimens into HGSOC subtypes and confirm previously reported subtype-associated features. EXPERIMENTAL DESIGN: Adopting two independent approaches, we derived and internally validated algorithms for subtype prediction using published gene expression data from 1,650 tumors. We applied resulting models to NanoString data on 3,829 HGSOCs from the Ovarian Tumor Tissue Analysis consortium. We further developed, confirmed, and validated a reduced, minimal gene set predictor, with methods suitable for a single-patient setting. RESULTS: Gene expression data were used to derive the predictor of high-grade serous ovarian carcinoma molecular subtype (PrOTYPE) assay. We established a de facto standard as a consensus of two parallel approaches. PrOTYPE subtypes are significantly associated with age, stage, residual disease, tumor-infiltrating lymphocytes, and outcome. The locked-down clinical grade PrOTYPE test includes a model with 55 genes that predicted gene expression subtype with >95% accuracy that was maintained in all analytic and biological validations. CONCLUSIONS: We validated the PrOTYPE assay following the Institute of Medicine guidelines for the development of omics-based tests. This fully defined and locked-down clinical grade assay will enable trial design with molecular subtype stratification and allow for objective assessment of the predictive value of HGSOC molecular subtypes in precision medicine applications.See related commentary by McMullen et al., p. 5271.Core funding for this project was provided by the National Institutes of Health (R01-CA172404, PI: S.J. Ramus; and R01-CA168758, PIs: J.A. Doherty and M.A.Rossing), the Canadian Institutes for Health Research (Proof-of-Principle I program, PIs: D.G.Huntsman and M.S. Anglesio), the United States Department of Defense Ovarian Cancer Research Program (OC110433, PI: D.D. Bowtell). A. Talhouk is funded through a Michael Smith Foundation for Health Research Scholar Award. M.S. Anglesio is funded through a Michael Smith Foundation for Health Research Scholar Award and the Janet D. Cottrelle Foundation Scholars program managed by the BC Cancer Foundation. J. George was partially supported by the NIH/National Cancer Institute award number P30CA034196. C. Wang was a Career Enhancement Awardee of the Mayo Clinic SPORE in Ovarian Cancer (P50 CA136393). D.G. Huntsman receives support from the Dr. Chew Wei Memorial Professorship in Gynecologic Oncology, and the Canada Research Chairs program (Research Chair in Molecular and Genomic Pathology). M. Widschwendter receives funding from the European Union’s Horizon 2020 European Research Council Programme, H2020 BRCA-ERC under Grant Agreement No. 742432 as well as the charity, The Eve Appeal (https://eveappeal.org.uk/), and support of the National Institute for Health Research (NIHR) and the University College London Hospitals (UCLH) Biomedical Research Centre. G.E. Konecny is supported by the Miriam and Sheldon Adelson Medical Research Foundation. B.Y. Karlan is funded by the American Cancer Society Early Detection Professorship (SIOP-06-258-01-COUN) and the National Center for Advancing Translational Sciences (NCATS), Grant UL1TR000124. H.R. Harris is 20 supported by the NIH/National Cancer Institute award number K22 CA193860. OVCARE (including the VAN study) receives support through the BC Cancer Foundation and The VGH+UBC Hospital Foundation (authors AT, BG, DGH, and MSA). The AOV study is supported by the Canadian Institutes of Health Research (MOP86727). The Gynaecological Oncology Biobank at Westmead, a member of the Australasian Biospecimen Network-Oncology group, was funded by the National Health and Medical Research Council Enabling Grants ID 310670 & ID 628903 and the Cancer Institute NSW Grants ID 12/RIG/1-17 & 15/RIG/1-16. The Australian Ovarian Cancer Study Group was supported by the U.S. Army Medical Research and Materiel Command under DAMD17-01-1-0729, The Cancer Council Victoria, Queensland Cancer Fund, The Cancer Council New South Wales, The Cancer Council South Australia, The Cancer Council Tasmania and The Cancer Foundation of Western Australia (Multi-State Applications 191, 211 and 182) and the National Health and Medical Research Council of Australia (NHMRC; ID199600; ID400413 and ID400281). BriTROC-1 was funded by Ovarian Cancer Action (to IAM and JDB, grant number 006) and supported by Cancer Research UK (grant numbers A15973, A15601, A18072, A17197, A19274 and A19694) and the National Institute for Health Research Cambridge and Imperial Biomedical Research Centres. Samples from the Mayo Clinic were collected and provided with support of P50 CA136393 (E.L.G., G.L.K, S.H.K, M.E.S.)

Educational challenges for children with cochlear implants

Children with severe to profound hearing loss who receive cochlear implants have an opportunity to develop the auditory skills that will assist them in lifelong learning. Although the auditory access provided by a cochlear implant can make this process easier, there remain a number of challenges that face a child with an implant, especially in the educational setting. Children with implants will still encounter acoustic challenges, academic challenges, attention challenges, associative challenges, and adjustment challenges. The knowledgeable speech and hearing professional who is aware of the potential of the implant while at the same time is cognizant of possible barriers to a child\u27s success in school, will assist the child is using the device to receive maximal benefit