A5059264212- Hemophilia Treatment and Research
- Platelet Disorders and Treatments
- Blood Coagulation and Thrombosis Mechanisms
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Complement system in diseases
- Cancer-related gene regulation
- Blood disorders and treatments
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Chronic Myeloid Leukemia Treatments
- Hemostasis and retained surgical items
- Blood groups and transfusion
- Hemoglobinopathies and Related Disorders
- Heparin-Induced Thrombocytopenia and Thrombosis
- Venous Thromboembolism Diagnosis and Management
- Immunodeficiency and Autoimmune Disorders
- Renal Diseases and Glomerulopathies
- Child Abuse and Related Trauma
- Systemic Lupus Erythematosus Research
- Iron Metabolism and Disorders
- Genomics and Rare Diseases
- Antiplatelet Therapy and Cardiovascular Diseases
- Central Venous Catheters and Hemodialysis
- Clinical Laboratory Practices and Quality Control
- Blood properties and coagulation
- melanin and skin pigmentation
Erasmus MC - Sophia Children’s Hospital
2022
Great Ormond Street Hospital
2012-2021
University College London
2012-2021
Great Ormond Street Hospital for Children NHS Foundation Trust
2012-2021
NIHR Great Ormond Street Hospital Biomedical Research Centre
2021
University Medical Center Groningen
2021
Cambridge University Hospitals NHS Foundation Trust
2016-2018
University of Cambridge
2018
University of Ottawa
2018
Children's Hospital of Eastern Ontario
2018
The guideline group was selected to be representative of UK-based medical experts. MEDLINE and EMBASE were searched systematically for publications in English, using the keywords: thrombotic thrombocytopenia purpura (TTP), ADAMTS13, plasma exchange (PEX) relevant key words related subsections this guideline. writing produced draft guideline, which subsequently revised by consensus members Haemostasis Thrombosis Task Force BCSH. then reviewed a sounding board British haematologists, BCSH...
Summary The guideline writing group was selected to be representative of UK‐based medical experts. MEDLINE systematically searched for publications in English up the Summer 2010 using key words platelet, platelet function testing and aggregometry. Relevant references generated from initial papers published guidelines/reviews were also examined. Meeting abstracts not included. produced draft guideline, which subsequently revised agreed by consensus. Further comment made members Haemostasis...
Thrombotic thrombocytopenic purpura (TTP) is an acute, rare, life-threatening disorder. This report presents the South East (SE) England registry for TTP, from April 2002 to December 2006, which included 176 patients and 236 acute episodes; 75% of were female 25% male, overall median age at presentation was 42 years. Mortality 8.5%, most cases died before treatment instigated. The main ethnic groups Caucasian (64%) Afro Caribbean (27%). Seventy-seven percent idiopathic, 5% congenital...
This document updates UK Haemophilia Centre Doctors Organization (UKHCDO) guidelines on the management of factor VIII/IX (FVIII/IX) inhibitors in congenital haemophilia (Hay et al, 2000, 2006). Acquired is excluded and will be covered separately. Most data apply to FVIII recommendations for FIX are sometimes extrapolated from this. Low titre defined as <5 Bethesda units (BU)/ml high ≥5 BU/ml. These targeted towards treaters UK. Not all may appropriate other countries with different health...
Twenty-seven children with severe haemophilia receiving regular prophylactic factor concentrate were evaluated to examine the overall effectiveness of prophylaxis in modern care. The median age at start was 6.2 years (range 1.3-15.9 years) and cumulative length follow-up 808 months (mean 30, rang 7-76 months). Nine patients required a central venous catheter for access (age range 1.3-5.2 years), eight boys could cannulate themselves 10 parents performed venepuncture. mean dose given time...
Recent reports have suggested that the incidence of inhibitors in haemophilia is highest those first exposed to factor VIII under 6 months age. In this study, we investigated inhibitor development children FVIII as neonates and also examined effect other genetic environmental variables. Three hundred forty-eight with severe A were investigated. Inhibitors developed 68 348 (20%), 34 (10%) high titre inhibitors. The relation initial exposure was: <1 month nine 35 (26%), 1-6 13 51 (25%), 6-12...
Prophylactic factor replacement, which prevents hemarthroses and thereby reduces the musculoskeletal disease burden in children with hemophilia A, requires frequent intravenous infusions (three to four times weekly).Kids A-LONG was a phase 3 open-label study evaluating safety, efficacy pharmacokinetics of longer-acting factor, recombinant VIII Fc fusion protein (rFVIIIFc), previously treated severe A (endogenous FVIII level < 1 IU dL(-1) [< 1%]).The enrolled 71 subjects. The starting...
Acquired coagulation inhibitors result from immune-mediated depletion or inhibition of a factor. Inhibitors are most commonly directed against factor VIII (FVIII) and von Willebrand (VWF) other factors only occasionally reported. Since the publication previous guidelines (Laffan et al, 2004; Pasi Hay 2006) substantial new data has been published on acquired FVIII inhibitors, necessitating updated guidelines. The rarity to means that limited information is available guide management treatment...
The effect of recombinant factor VIII (rFVIII) brand on inhibitor development was investigated in all 407 severe hemophilia A previously untreated patients born the United Kingdom (UK) between 1 January 2000 and 31 December 2011. Eighty-eight (22%) had been RODIN study. Information extracted from National Haemophilia Database. Because exposure days (EDs) were not known for some patients, time first treatment used as a surrogate rFVIII exposure. An developed 118 (29%) 60 high 58 low titer,...
The incidence of FIX inhibitors in severe hemophilia B (SHB) is not well defined. Frequencies 3-5% have been reported but most studies to date were small, including patients with different severities, and without prospective follow-up for inhibitor incidence. Study objective was investigate SHB followed up 500 exposure days (ED), the frequency allergic reactions, relationship genotypes. Consecutive previously untreated (PUPs) enrolled into PedNet cohort included. Detailed data collected...
As a result of the new treatment paradigm that haemophilia community will face with availability novel (non-factor) therapies, an updated consensus on ITI recommendations and inhibitor management strategies is needed. The Future Immunotolerance Treatment (FIT) group was established to contemplate, determine recommend best options for patients A inhibitors. Despite considerable success emicizumab in patients, FIT still sees importance eradicating However, other non-factor therapies future...