James, Paula - Queen's University - Department of Medicine

个人简介

Dr. Paula James is a Professor in the Department of Medicine, with cross-appointments to Pathology & Molecular Medicine and Pediatrics. She joined the Faculty in July of 2004 and is a Clinician Scientist with 75% protected time for research. Dr. James is a Principal Investigator in the Clinical and Molecular Hemostasis Research Group at Queen’s University with an active basic and clinical research program investigating the genetic basis of inherited bleeding disorders as well as the quantitation of bleeding symptoms. Her group is comprised of post-doctoral fellows, graduate students and well as research associates. In recent years, activities have included validating and publishing the Condensed MCMDM-1VWD Bleeding Questionnaire, which has gained international recognition as well as optimizing a protocol for culturing BOEC (Blood Outgrowth Endothelial Cells) from patients with both inherited and acquired diseases. In addition to her research activities, Dr. James is the Medical Director of the Southeastern Ontario Inherited Bleeding Disorders Program and its aligned Women and Bleeding Disorders Clinic. She was awarded “Researcher of the Year” by the National Hemophilia Foundation (US organization) in 2011, and the Mihran and Mary Basmajian Award for Excellence in Health Research by the Faculty of Health Sciences at Queen’s University in 2012.

研究领域

The clinical impact of inherited bleeding disorders including disease prevalence of quantitative assessments of bleeding The use of bleeding scores as a screening tool for inherited bleeding disorders and to assess disease severity The underlying pathophysiology of inherited bleeding disorders at a cellular and molecular level Current Models BOEC (Blood Outgrowth Endothelial Cells) Active/Future Studies The use of BOEC to understand angiodysplasia in von Willebrand disease (VWD) and to investigate potential novel treatments A national study evaluating abnormal bleeding in female carriers of hemophilia Identification of undiagnosed individuals affected with an inherited bleeding disorder using a social media campaign

近期论文

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Deforest M, Grabell J, Albert S, Young J, Tuttle A, Hopman WM, James PD. Generation and Optimization of the Self-Administered Bleeding Assessment Tool (Self-BAT) and its Validation as a Screening Test for von Willebrand Disease (VWD). Haemophilia July 14, 2015 published as an Early View online; DOI:10.1111/hae.12747 Rydz N, Swystun L, Notley C, Paterson A, Riches J, Boonyarat B, Montgomery RR, James PD, Lillicrap D. The C-type lectin receptor CLEC4M (L-SIGN) binds to, internalizes and contributes to the clearance of von WIllebrand factor and variation in plasma von Willebrand factor levels. Blood 2013 Jun 27; 121(26): 5228-37. PMID: 23529928 Bowman M, Tuttle A, Notley C, Brown C, Tinlin S, Deforest M, Leggo J, Blanchette VS, Lillicrap D, James P for the Association of Hemophilia Clinic Directors of Canada. The Genetics of Canadian Type 3 von WIllebrand Disease (VWD): Further Evidence for Co-dominant Inheritance of Type 3 VWD Alleles. J Thromb Haemost 2013 Mar; 11(3): 512-20. PMID 23311757 Bowman M, Mundell G, Grabell J, Hopman W, Rapson D, Lillicrap D, James P. Generation and Validation of the Condensed MCMDM1-VWD Bleeding Questionnaire. J Thromb Haemost 2008; 6: 2062-6. PMID: 18983516