Toddrichard3740

RESULTS For isocitrate dehydrogenase (IDH) wild-type lower-grade gliomas, tumor size (hazard ratio, 3.82; 95% CI, 1.94-7.75; P  less then  .001), number of involved lobes (hazard ratio, 1.70; 95% CI, 1.28-2.27; P  less then  .001), hydrocephalus (hazard ratio, 4.43; 95% CI, 1.12-17.54; P = .034), midline shift (hazard ratio, 1.16; 95% CI, 1.03-1.30; P = .013), margins (P = .031), and contrast enhancement (hazard ratio, 0.34; 95% CI, 0.13-0.90; P = .030) were associated with overall survival. For IDH-mutant 1p/19q-codeleted lower-grade gliomas, tumor size (hazard ratio, 2.85; 95% CI, 1.06-7.70; P = .039) and ependymal extension (hazard ratio, 6.34; 95% CI, 1.07-37.59; P = .042) were associated with overall survival. CONCLUSIONS MR imaging metrics offers prognostic information for patients with lower-grade gliomas within molecularly defined classes, with the greatest prognostic value for IDH wild-type lower-grade gliomas. © 2020 by American Journal of Neuroradiology.BACKGROUND AND PURPOSE Though CT is a highly calibrated imaging modality, head CT is typically interpreted qualitatively. Our aim was to initiate the establishment of a reference quantitative database for clinical head CT. MATERIALS AND METHODS An automated segmentation algorithm was developed and applied to 354 clinical head CT scans with radiographically normal findings (ages, 18-101 years; 203 women) to measure brain volume, brain parenchymal fraction, brain radiodensity, and brain parenchymal radiomass. Brain parenchymal fraction was modeled using quantile regression analysis. RESULTS Brain parenchymal fraction is highly correlated with age (R 2 = 0.908 for men and 0.950 for women), with 11% overall brain volume loss in the adult life span (1%/year from 20 to 50 years and 2%/year after 50 years of age). Third-order polynomial quantile regression curves for brain parenchymal fraction were rationalized and statistically validated. Total brain parenchymal radiodensity shows a decline as a function of age (14.9% for men, 14.7% for women; slopes not significantly different, P = .760). Age-related loss of brain radiomass (the product of volume and radiodensity) is approximately 20% for both sexes, significantly greater than the loss of brain volume (P  less then  .001). CONCLUSIONS An automated segmentation algorithm has been developed and applied to clinical head CT images to initiate the development of a reference database for quantitative brain CT imaging. Such a database can be subject to quantile regression analysis to stratify patient brain CT scans by metrics such as brain parenchymal fraction, radiodensity, and radiomass, to aid in the identification of statistical outliers and lend quantitative assessment to image interpretation. © 2020 by American Journal of Neuroradiology.Proteus syndrome is mosaic disorder that can cause progressive postnatal overgrowth of nearly any organ or tissue. To date, Proteus syndrome has been exclusively associated with the mosaic c.49G>A p.(Glu17Lys) pathogenic variant in AKT1, a variant that is also present in many cancers. Here we describe an individual with severe Proteus syndrome who died at 7.5 years of age from combined parenchymal and restrictive pulmonary disease. Remarkably, this individual was found to harbor a mosaic c.49_50delinsAG p.(Glu17Arg) variant in AKT1 at a variant allele fraction that ranged from less then 0.01 to 0.46 in fibroblasts established from an overgrown digit. This variant was demonstrated to be constitutively activating by phosphorylation of AKT(S473). These data document allelic heterogeneity for Proteus syndrome. We recommend that individuals with a potential clinical diagnosis of Proteus syndrome who are negative for the p.(Glu17Lys) variant be tested for other variants in AKT1. Cold Spring Harbor Laboratory Press.Management guidelines assume that results from clinical trials can be generalised, although seldom is data available to test this assumption. We aimed to determine the proportion of patients commencing tumour necrosis factor inhibition (TNFi) who would have been eligible for relevant clinical trials, and whether treatment response differs between these groups and the trials themselves. The British Society for Rheumatology Biologics Register for Ankylosing Spondylitis (BSRBR-AS) recruited a real-world cohort of TNFi-naïve spondyloarthritis patients with data collection from clinical records and patient questionnaires. Participant characteristics were extracted from trials identified from a recent Health Technology Assessment of TNFi for ankylosing spondylitis/non-radiographic axial spondyloarthritis. Descriptive statistics were used to determine the differences, including treatment response, between BSRBR-AS participants who would/would not have been eligible for the clinical trials and with trial participants by BMJ.BACKGROUND Rheumatic and musculoskeletal immune-related adverse events (irAEs) are observed in about 10% of patients with cancer receiving checkpoint inhibitors (CPIs). Given the recent emergence of these events and the lack of guidance for rheumatologists addressing them, a European League Against Rheumatism task force was convened to harmonise expert opinion regarding their identification and management. METHODS First, the group formulated research questions for a systematic literature review. Then, based on literature and using a consensus procedure, 4 overarching principles and 10 points to consider were developed. read more RESULTS The overarching principles defined the role of rheumatologists in the management of irAEs, highlighting the shared decision-making process between patients, oncologists and rheumatologists. The points to consider inform rheumatologists on the wide spectrum of musculoskeletal irAEs, not fulfilling usual classification criteria of rheumatic diseases, and their differential diagnoses. Earl. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.INTRODUCTION The UK Department of Health have targeted a reduction in stillbirth by 50% by 2025; to achieve this, the first version of the Saving Babies' Lives Care Bundle (SBLCB) was developed by NHS England in 2016 to improve four key areas of antenatal and intrapartum care. Clinical practice guidelines are a key means by which quality improvement initiatives are disseminated to front-line staff. METHODS Seventy-five clinical practice guidelines covering the four areas of antenatal and intrapartum care in the first version of SBLCB were obtained from 19 maternity providers. The content and quality of guidelines were evaluated using the Appraisal of Guidelines for Research and Evaluation (AGREE II) tool. Maternity health professionals in participating organisations were invited to participate in an anonymous survey to determine perceptions toward and experiences of the use of clinical practice guidelines using a series of Likert scales. RESULTS Unit guidelines showed considerable variation in quality with median scores of 50%-58%.