A5055945057- Hemophilia Treatment and Research
- Platelet Disorders and Treatments
- Blood Coagulation and Thrombosis Mechanisms
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Venous Thromboembolism Diagnosis and Management
- Hemostasis and retained surgical items
- Atrial Fibrillation Management and Outcomes
- Blood groups and transfusion
- COVID-19 Clinical Research Studies
- Heparin-Induced Thrombocytopenia and Thrombosis
- Virus-based gene therapy research
- X-ray Diffraction in Crystallography
- Crystallization and Solubility Studies
- Cholesterol and Lipid Metabolism
- Blood disorders and treatments
- Long-Term Effects of COVID-19
- Chronic Myeloid Leukemia Treatments
- CAR-T cell therapy research
- Orthopedic Infections and Treatments
- CRISPR and Genetic Engineering
- COVID-19 and healthcare impacts
- Crystallography and molecular interactions
- Complement system in diseases
- Plant biochemistry and biosynthesis
St. James's Hospital
2016-2025
Trinity College Dublin
2001-2024
Sheffield Teaching Hospitals NHS Foundation Trust
2024
Essen University Hospital
2024
University College London
2003-2022
Whittington Hospital
2022
Carolinas Medical Center
2021
Elekta (Sweden)
2016
Boston Children's Hospital
2014
University College Cork
2012-2013
Although the pathophysiology underlying severe COVID19 remains poorly understood, accumulating data suggest that a lung-centric coagulopathy may play an important role. Elevated D-dimer levels which correlated inversely with overall survival were recently reported in Chinese cohort studies. Critically however, ethnicity has major effects on thrombotic risk, 3-4-fold lower risk compared to Caucasians and significantly higher African-Americans. In this study, we investigated Caucasian...
The objective of this document is to guide diagnosis and management patients with rare coagulation disorders (RCD). This replaces the 2004 UK Haemophilia Centre Doctors' Organization (UKHCDO) guideline (Bolton-Maggs et al, 2004a). RCD are here defined as monogenic bleeding caused by deficiency a soluble factor or factors, other than von Willebrand disease (VWD), A B. described in include heritable deficiencies fibrinogen, prothrombin, (F) V, FVII, FX, FXI FXIII, combined FV FVIII vitamin...
Moderate-to-severe hemophilia B is treated with lifelong, continuous coagulation factor IX replacement to prevent bleeding. Gene therapy for aims establish sustained activity, thereby protecting against bleeding without burdensome replacement.
BackgroundPersistent fatigue, breathlessness, and reduced exercise tolerance have been reported following acute COVID‐19 infection. Although immuno‐thrombosis has implicated in pathogenesis, the biological mechanisms underpinning long COVID remain unknown. We hypothesized that pulmonary microvascular may be important this context.MethodsOne hundred fifty patients were reviewed at St James's Hospital Dublin between May September 2020 a median of 80.5 (range 44–155) days after initial...
BackgroundConsistent with fulminant endothelial cell activation, elevated plasma von Willebrand factor (VWF) antigen levels have been reported in patients COVID‐19. The multimeric size and function of VWF are normally regulated through A Disintegrin And Metalloprotease ThrombSpondin Motif type 1 motif, member 13 (ADAMTS‐13)‐‐mediated proteolysis.ObjectivesThis study investigated the hypothesis that ADAMTS‐13 regulation multimer distribution may be impaired severe acute respiratory...
BackgroundProlonged recovery is common after acute SARS-CoV-2 infection; however, the pathophysiological mechanisms underpinning Long COVID syndrome remain unknown. VWF/ADAMTS-13 imbalance, dysregulated angiogenesis, and immunothrombosis are hallmarks of COVID-19. We hypothesized that imbalance persists in convalescence together with endothelial cell (EC) activation angiogenic disturbance. Additionally, we postulate ongoing immune dysfunction may be linked to sustained EC coagulation...
Summary Vaccine‐induced immune thrombotic thrombocytopenia (VITT) is a novel entity that emerged in March 2021 following reports of unusual thrombosis after ChAdOx1 nCoV‐19, (AstraZeneca) vaccination. Following the recognition this syndrome, multiple consensus guidelines have been released to risk stratify patients presenting with possible symptoms nCoV‐19 All rapidly identify VITT complete triad thrombocytopenia, and elevated D‐dimers However, earlier associated symptoms, clinical...
BACKGROUND: Recombinant FVIIa (rFVIIa/NovoSeven) is a novel hemostatic agent originally developed to treat patients with hemophilia who had inhibitors. Several case reports have suggested that rFVIIa may be effective in treating without pre‐existing bleeding disorder uncontrolled bleeding. STUDY DESIGN AND METHODS: Data on the efficacy and safety of treatment massive hemorrhage were obtained retrospectively from NovoSeven extended‐use data collection system. RESULTS: A total 40 received for...
Abstract Introduction The COVID‐19 pandemic caused an unprecedented impact to haemophilia healthcare delivery. In particular, rapid implementation of telehealth solutions was required ensure continued access comprehensive care. Aims To explore patient and provider (HCP) experience in a European Haemophilia Comprehensive Care Centre. Method A systematic evaluation performed survey HCP compare clinical activity levels with in‐person attendances. Results Public health measures implemented March...
Abstract There is significant ongoing debate regarding type 1 von Willebrand disease (VWD) defintion. Previous guidelines recommended patients with factor (VWF) levels <30 IU/dL be diagnosed VWD, whereas bleeding and VWF from 30 to 50 low VWF. To elucidate the relationship between VWD in context of age-induced increases levels, we combined data sets 2 national cohort studies: 162 Low Ireland Cohort (LoVIC) 403 The Netherlands (WiN) studies. In 47% participants, remained despite...
Factor XI (FXI) is the zymogen of a serine protease enzyme in intrinsic pathway blood coagulation and an important factor creation stable fibrin clot. Deficiency FXI leads to injury-related bleeding disorder remarkable for lack correlation between symptoms coagulant activity (FXI:C). The protein composed five domains: four tandem repeat domains ∼80 residues known as Apple (Ap) domains, catalytic (Sp) domain. A total 65 mutations throughout gene (F11) have been reported deficient patients. An...
Previous studies have reported marked interindividual variation in factor VIII (FVIII) clearance patients with hemophilia (PWH) and proposed a number of factors that influence this heterogeneity.To investigate the importance rates endogenous von Willebrand (VWF) compared those other FVIII half-life modifiers adult PWH.The recombinant was determined cohort 61 PWH. A range assessed (including plasma VWF:antigen VWF propeptide levels; VWF-FVIII binding capacity; ABO blood group; nonneutralizing...
Previous reports have highlighted that some low VWF patients with significant bleeding were diagnosed based upon an isolated but persistent reduction in plasma activity levels the 30-50 IU/dL range. These had VWF:Ag > 50 and thus 'qualitative' rather than 'quantitative' VWF. Although clinical importance of functional defects type 2 VWD is well recognized, translational implications mild qualitative (low VWF-QL) not been defined. To address this clinically important question, we...
We assessed the prevalence of von Willebrand's disease (VWD) in patients with objectively confirmed dysfunctional uterine bleeding. A case-control study was designed to include 38 bleeding and age-matched controls normal menstrual blood loss (MBL). Menorrhagia defined as a mean MBL greater than 80 ml on three consecutive menses measured by alkali haematin method. Willebrand factor antigen, activity (VWF:Ac) VIII:C were serial venous samples 1 week apart. VWD diagnosed five (13%) menorrhagia...
Summary. Factor XI (FXI) deficiency is associated with bleeding after invasive procedures. Risks of human plasma‐derived FXI replacement products include transfusion transmitted infection, thrombosis and fluid overload. This study was designed to test the hypothesis that recombinant factor VIIa (rFVIIa) an effective haemostatic agent in patients undergoing surgery. Fourteen deficient [five severely (FXI:C <20 U dL −1 ) nine partially 20–70 ] received rFVIIa prevent surgical during five...