Publications
347 results found
McLintock C, Pabinger I, Bauer KA, et al., 2016, International Society on Thrombosis and Haemostasis core curriculum project: core competencies in clinical thrombosis and hemostasis, Journal of Thrombosis and Haemostasis, Vol: 14, Pages: 3-27, ISSN: 1538-7933
BackgroundThe International Society on Thrombosis and Haemostasis (ISTH) identified the need for an international core curriculum on thrombosis and hemostasis for its society members and the larger thrombosis and hemostasis community.AimsThe current research sought consensus on the core competencies required by medical doctors who are ready to practise as independent clinical specialists in thrombosis and hemostasis with the aim of developing a core clinical curriculum for specialists in the field.MethodA draft list of competencies was developed by the Working Group and formed the basis of an online survey. ISTH members and the larger thrombosis and hemostasis community were asked to rate the importance of each competency, on a Likert scale, for clinical specialists in thrombosis and hemostasis.ResultsThere were a total of 644 responses to the online survey with broad geographical representation. There was general agreement on what level of competency would be required for clinical specialists in thrombosis and hemostasis at the specified level of training.ConclusionsUsing the survey to gain consensus on the level of competency required by clinical specialists in the field of thrombosis and hemostasis enabled the development of a core clinical curriculum that has been endorsed by the ISTH Council. The curriculum will offer a framework and international reference that will be used by the society, by national and regional organizations, and for further research.
Laffan M, 2016, New products for the treatment of haemophilia, BRITISH JOURNAL OF HAEMATOLOGY, Vol: 172, Pages: 23-31, ISSN: 0007-1048
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- Citations: 14
Laffan MA, Pasi KJ, 2016, Haemophilia and Von Willebrand Disease, POSTGRADUATE HAEMATOLOGY, 7TH EDITION, Editors: Hoffbrand, Higgs, Keeling, Mehta, Publisher: JOHN WILEY & SONS LTD, Pages: 715-732, ISBN: 978-1-118-85432-7
Thompson J, Mkandawire C, Chakravorty S, et al., 2015, A pilot study of assessing cognition in children with sickle cell disease using a new software package the cogstate battery, 57th Annual Meeting of the American Society of Hematology, Publisher: American Society of Hematology, ISSN: 0006-4971
Nowak AA, Laffan M, McKinnon TAJ, 2015, The N-linked glycans of ADAMTS13 are critical determinants of conformation and proteolytic activity, 57th Annual Meeting of the American Society of Hematology, Publisher: American Society of Hematology, ISSN: 0006-4971
Wong CL, Gerrard G, Adamowicz-Brice M, et al., 2015, Targeted sequencing of sorted peripheral blood cells reveals novel germline and somatic variants in the polymorphonuclear and mononuclear cells of patients with essential thrombocythemia, polycythemia vera and primary myelofibrosis, 57th Annual Meeting of the American Society of Hematology, Publisher: American Society of Hematology, Pages: 5213-5213, ISSN: 0006-4971
Wong CL, Ma B, Gerrard G, et al., 2015, Gene expression profiling of sorted peripheral blood cells using microarray and next generation sequencing reveals distinct molecular signatures in the polymorphonuclear and mononuclear cells of patients with polycythemia vera and primary myelofibrosis, 57th Annual Meeting of the American Society of Hematology, Publisher: American Society of Hematology, Pages: 5201-5201, ISSN: 0006-4971
Wong CL, Ma B, Adamowicz-Brice M, et al., 2015, DNA Methylation Profiling of Sorted Peripheral Blood Cells Using Microarray and Next Generation Sequencing Reveals Distinct Molecular Signatures in the Polymorphonuclear and Mononuclear Cells of Patients with Essential Thrombocythemia, Polycythemia Vera and Primary Myelofibrosis, 57th Annual Meeting of the American Society of Hematology, Publisher: American Society of Hematology, ISSN: 0006-4971
Shovlin CL, Basel C, Santhirapala V, et al., 2015, Moderate iron deficiency doubles the risk of ischemic stroke risk for patients with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations, Publisher: SPRINGER, Pages: 539-540, ISSN: 0969-6970
Khan E, Ambrose N, Stanford M, et al., 2015, Microparticles (MPs) Derived from Cell Plasma Membranes Are Increased in Behcet's Syndrome (BS) and a Low Ratio of Tissue Factor Pathway Inhibitor Positive Mps to Tissue Factor Positive Mps (TFPI/TF) Is Associated with Thrombosis, ARTHRITIS & RHEUMATOLOGY, Vol: 67, ISSN: 2326-5191
Watson HG, Keeling DM, Laffan M, et al., 2015, Guideline on aspects of cancer-related venous thrombosis, BRITISH JOURNAL OF HAEMATOLOGY, Vol: 170, Pages: 640-648, ISSN: 0007-1048
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- Citations: 112
Nowak AA, de Groot R, Laffan MA, et al., 2015, N-linked glycosylation is a modulator of ADAMTS13 expression, structure and function, Journal of Thrombosis and Haemostasis, Vol: 13, Pages: 64-64, ISSN: 1538-7933
Schreiber K, Sciascia S, Breen K, et al., 2015, Proposed trial: hypatia - a prospective randomised controlled trial of hydroxychoroquine versus placebo during pregnancy in women with antiphospholipid antibodies, XXV Congress of the International Society on Thrombosis and Haemostasis, Publisher: Wiley, Pages: 281-281, ISSN: 1538-7836
Wong CL, Gerrard G, Norziha ZA, et al., 2015, JAK2V617F MUTATION IN COMBINATION WITH ASXL1, DNMT3A, TET2, U2AF1 AND RUNX1 VARIANTS IS ASSOCIATED WITH SEVERE CLINICAL PHENOTYPES IN PRIMARY MYELOFIBROSIS, 20th Congress of European-Hematology-Association, Publisher: FERRATA STORTI FOUNDATION, Pages: 531-531, ISSN: 0390-6078
Chowdary P, Lethagen S, Friedrich U, et al., 2015, Safety and pharmacokinetics of anti-TFPI antibody (concizumab) in healthy volunteers and patients with hemophilia: a randomized first human dose trial, JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Vol: 13, Pages: 743-754, ISSN: 1538-7933
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- Citations: 170
Westbury SK, Turro E, Greene D, et al., 2015, Human phenotype ontology annotation and cluster analysis to unravel genetic defects in 707 cases with unexplained bleeding and platelet disorders, Genome Medicine, Vol: 7, ISSN: 1756-994X
BackgroundHeritable bleeding and platelet disorders (BPD) are heterogeneous and frequently have an unknown genetic basis. The BRIDGE-BPD study aims to discover new causal genes for BPD by high throughput sequencing using cluster analyses based on improved and standardised deep, multi-system phenotyping of cases.MethodsWe report a new approach in which the clinical and laboratory characteristics of BPD cases are annotated with adapted Human Phenotype Ontology (HPO) terms. Cluster analyses are then used to characterise groups of cases with similar HPO terms and variants in the same genes.ResultsWe show that 60% of index cases with heritable BPD enrolled at 10 European or US centres were annotated with HPO terms indicating abnormalities in organ systems other than blood or blood-forming tissues, particularly the nervous system. Cases within pedigrees clustered closely together on the bases of their HPO-coded phenotypes, as did cases sharing several clinically suspected syndromic disorders. Cases subsequently found to harbour variants in ACTN1 also clustered closely, even though diagnosis of this recently described disorder was not possible using only the clinical and laboratory data available to the enrolling clinician.ConclusionsThese findings validate our novel HPO-based phenotype clustering methodology for known BPD, thus providing a new discovery tool for BPD of unknown genetic basis. This approach will also be relevant for other rare diseases with significant genetic heterogeneity.
Dharmarajah B, McKinnon TA, Keravnou C, et al., 2015, Thrombus dissolution using contrast enhanced ultrasound in an in-vitro model of acute deep vein thrombosis, Annual Meeting of the Society-of-Academic-and-Research-Surgery (SARS(, Publisher: WILEY-BLACKWELL, Pages: 37-38, ISSN: 0007-1323
Smith K, Starke RD, Mckinnon TAJ, et al., 2015, RECIPROCAL REGULATION OF ANGIOPOIETIN 2 & VON WILLEBRAND FACTOR EXPRESSION IN ENDOTHELIAL CELLS, Joint Meeting of the European-Society-for-Microcirculation (ESM) and European-Vascular-Biology-Organisation (EVBO), Publisher: KARGER, Pages: 51-52, ISSN: 1018-1172
Laffan M, 2015, Specific treatment for cancer-associated thrombosis, FUTURE ONCOLOGY, Vol: 11, Pages: 3061-3063, ISSN: 1479-6694
Chion A, O'Sullivan J, Bergsson G, et al., 2014, N-Linked Glycans within the A1A2A3 Domains of VWF Play a Critical Role in Modulating Macrophage-Mediated Clearance, BLOOD, Vol: 124, ISSN: 0006-4971
Iqbal MB, Johns M, Cao J, et al., 2014, PARP-14 combines with tristetraprolin in the selective posttranscriptional control of macrophage tissue factor expression, Blood, Vol: 124, Pages: 3646-3655, ISSN: 0006-4971
Tissue factor (TF) (CD142) is a 47 kDa transmembrane cell surface glycoprotein that triggers the extrinsic coagulation cascade and links thrombosis with inflammation. Although macrophage TF expression is known to be regulated at the RNA level, very little is known about the mechanisms involved. Poly(adenosine 5′-diphosphate [ADP]-ribose)-polymerase (PARP)-14 belongs to a family of intracellular proteins that generate ADP-ribose posttranslational adducts. Functional screening of PARP-14–deficient macrophages mice revealed that PARP-14 deficiency leads to increased TF expression and functional activity in macrophages after challenge with bacterial lipopolysaccharide. This was related to an increase in TF messenger RNA (mRNA) stability. Ribonucleoprotein complex immunoprecipitation and biotinylated RNA pull-down assays demonstrated that PARP-14 forms a complex with the mRNA-destabilizing protein tristetraprolin (TTP) and a conserved adenylate-uridylate-rich element in the TF mRNA 3′ untranslated region. TF mRNA regulation by PARP-14 was selective, as tumor necrosis factor (TNF)α mRNA, which is also regulated by TTP, was not altered in PARP-14 deficient macrophages. Consistent with the in vitro data, TF expression and TF activity, but not TNFα expression, were increased in Parp14−/− mice in vivo. Our study provides a novel mechanism for the posttranscriptional regulation of TF expression, indicating that this is selectively regulated by PARP-14.
Berntorp E, Dolan G, Hermans C, et al., 2014, Pharmacokinetics, phenotype and product choice in haemophilia B: how to strike a balance?, HAEMOPHILIA, Vol: 20, Pages: 1-11, ISSN: 1351-8216
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- Citations: 10
Laffan MA, Lester W, O'Donnell JS, et al., 2014, The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology, BRITISH JOURNAL OF HAEMATOLOGY, Vol: 167, Pages: 453-465, ISSN: 0007-1048
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- Citations: 225
Chen L, Kostadima M, Martens JHA, et al., 2014, Transcriptional diversity during lineage commitment of human blood progenitors, SCIENCE, Vol: 345, Pages: 1580-+, ISSN: 0036-8075
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- Citations: 185
Patel A, Laffan MA, Waheed U, et al., 2014, Randomised trials of human albumin for adults with sepsis: systematic review and meta-analysis with trial sequential analysis of all-cause mortality, British Medical Journal, Vol: 349, ISSN: 0959-8138
Objective To assess the efficacy and safety of pooled human albumin solutions as part of fluid volume expansion and resuscitation (with or without improvement of baseline hypoalbuminaemia) in critically unwell adults with sepsis of any severity.Design Systematic review and meta-analysis of randomised clinical trials, with trial sequential analysis, subgroup, and meta-regression analyses.Data sources PubMed, PubMed Central, Web of Science (includes Medline, Conference Proceedings Citation Index, Data Citation Index, Chinese Science Citation Database, CAB abstracts, Derwent Innovations Index), OvidSP (includes Embase, Ovid Medline, HMIC, PsycINFO, Maternity and Infant Care, Transport Database), Cochrane Library, clinicaltrials.gov, controlled-trials.com, online material, relevant conference proceedings, hand searching of reference lists, and contact with authors as necessary.Eligibility criteria Prospective randomised clinical trials of adults with sepsis of any severity (with or without baseline hypoalbuminaemia) in critical or intensive care who received pooled human albumin solutions as part of fluid volume expansion and resuscitation (with or without improvement of hypoalbuminaemia) compared with those who received control fluids (crystalloid or colloid), were included if all-cause mortality outcome data were available. No restriction of language, date, publication status, or primary study endpoint was applied.Data extraction Two reviewers independently assessed articles for inclusion, extracted data to assess risk of bias, trial methods, patients, interventions, comparisons, and outcome. The relative risk of all-cause mortality was calculated using a random effects model accounting for clinical heterogeneity.Primary outcome measure All-cause mortality at final follow-up.Results Eighteen articles reporting on 16 primary clinical trials that included 4190 adults in critical or intensive care with sepsis, severe sepsis, or septic shock. A median of 70.0 g daily of p
Astermark J, Dolan G, Hilberg T, et al., 2014, Managing Haemophilia for Life: 4th Haemophilia Global Summit, HAEMOPHILIA, Vol: 20, Pages: 1-20, ISSN: 1351-8216
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- Citations: 4
Shovlin CL, Chamali B, Santhirapala V, et al., 2014, Ischaemic strokes in patients with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia: associations with iron deficiency and platelets., PLOS One
Background: Pulmonary first pass filtration of particles marginally exceeding ~7 µm (the size of a red blood cell) is used routinely in diagnostics, and allows cellular aggregates forming or entering the circulation in the preceding cardiac cycle to lodge safely in pulmonary capillaries/arterioles. Pulmonary arteriovenous malformations compromise capillary bed filtration, and are commonly associated with ischaemic stroke. Cohorts with CT-scan evident malformations associated with the highest contrast echocardiographic shunt grades are known to be at higher stroke risk. Our goal was to identify within this broad grouping, which patients were at higher risk of stroke.Methodology: 497 consecutive patients with CT-proven pulmonary arteriovenous malformations due to hereditary haemorrhagic telangiectasia were studied. Relationships with radiologically-confirmed clinical ischaemic stroke were examined using logistic regression, receiver operating characteristic analyses, and platelet studies.Principal Findings: Sixty-one individuals (12.3%) had acute, non-iatrogenic ischaemic clinical strokes at a median age of 52 (IQR 41–63) years. In crude and age-adjusted logistic regression, stroke risk was associated not with venous thromboemboli or conventional neurovascular risk factors, but with low serum iron (adjusted odds ratio 0.96 [95% confidence intervals 0.92, 1.00]), and more weakly with low oxygen saturations reflecting a larger right-to-left shunt (adjusted OR 0.96 [0.92, 1.01]). For the same pulmonary arteriovenous malformations, the stroke risk would approximately double with serum iron 6 µmol/L compared to mid-normal range (7–27 µmol/L). Platelet studies confirmed overlooked data that iron deficiency is associated with exuberant platelet aggregation to serotonin (5HT), correcting following iron treatment. By MANOVA, adjusting for participant and 5HT, iron or ferritin explained 14% of the variance in log-transformed aggregation-rate (p =
Shapiro SE, Nowak AA, Wooding C, et al., 2014, The von Willebrand factor predicted unpaired cysteines are essential for secretion, JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Vol: 12, Pages: 246-254, ISSN: 1538-7933
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- Citations: 14
Nowak AA, McKinnon TAJ, Hughes JM, et al., 2014, The O-linked glycans of human von Willebrand factor modulate its interaction with ADAMTS-13, JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Vol: 12, Pages: 54-61, ISSN: 1538-7933
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- Citations: 13
Laffan M, 2014, Fibrinogen deficiency, TEXTBOOK OF HEMOPHILIA, 3RD EDITION, Editors: Lee, Berntorp, Hoots, Publisher: BLACKWELL SCIENCE PUBL, Pages: 445-451, ISBN: 978-1-118-39824-1
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