A5045147396- Hemoglobinopathies and Related Disorders
- Blood groups and transfusion
- Iron Metabolism and Disorders
- Erythrocyte Function and Pathophysiology
- Folate and B Vitamins Research
- Blood transfusion and management
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Neonatal Health and Biochemistry
- Heme Oxygenase-1 and Carbon Monoxide
- Blood properties and coagulation
- Blood donation and transfusion practices
- Chronic Disease Management Strategies
- Blood Coagulation and Thrombosis Mechanisms
- Muscle and Compartmental Disorders
- Cardiac Imaging and Diagnostics
- Multiple Myeloma Research and Treatments
- Blood disorders and treatments
- Phagocytosis and Immune Regulation
- Aluminum toxicity and tolerance in plants and animals
- Parvovirus B19 Infection Studies
- Advanced MRI Techniques and Applications
- Meta-analysis and systematic reviews
- Autopsy Techniques and Outcomes
- Fetal and Pediatric Neurological Disorders
- Acute Ischemic Stroke Management
NHS Blood and Transplant
2015-2024
University College Hospital
2014-2024
University College London Hospitals NHS Foundation Trust
2015-2024
University College London
2015-2024
University of Cambridge
2023
National Health Service
2021
Great Ormond Street Hospital
2008-2017
Hospital for Sick Children
2017
The University of Western Australia
2017
Northwestern University
1991
Purpose To explore the use and reproducibility of magnetic resonance‐derived myocardial T 1 mapping in patients with iron overload. Materials Methods The research received ethics committee approval all provided written informed consent. This was a prospective study 88 67 healthy volunteers. Thirty‐five underwent repeat scanning for reproducibility. used shortened modified Look–Locker inversion recovery sequence (ShMOLLI) second, confirmatory MOLLI group. 2 * performed using commercially...
Abstract Each year, blood transfusions save millions of lives. However, under current blood-matching practices, sensitization to non–self-antigens is an unavoidable adverse side effect transfusion. We describe a universal donor typing platform that could be adopted by services worldwide facilitate extended policy and reduce rates. This DNA-based test capable simultaneously most clinically relevant red cell (RBC), human platelet (HPA), leukocyte (HLA) antigens. Validation was performed, using...
Abstract Background Sickle cell disease (SCD) is a devastating, multisystemic disorder that affects millions of people worldwide. The earliest clinical manifestations SCD can affect infants as young 6 months age, and pediatric patients are at risk for acute life‐threatening complications. Early intervention with treatments target the underlying pathophysiological mechanism SCD, sickle hemoglobin (HbS) polymerization, expected to slow progression circumvent disease‐associated morbidity...
Summary With the developing COVID‐19 pandemic, patients with inherited anaemias require specific advice regarding isolation and changes to usual treatment schedules. The National Haemoglobinopathy Panel (NHP) has issued guidance on care of sickle cell disease, thalassaemia, Diamond Blackfan anaemia (DBA), congenital dyserythropoietic (CDA), sideroblastic anaemia, pyruvate kinase deficiency other red enzyme membrane disorders. Cascading accurate information for clinicians is paramount...
Real-time national survey of COVID-19 in hemoglobinopathy and rare inherited anemia patientsFaced with the rapidly evolving pandemic, March 2020 UK Government advocated strict self-isolation ('shielding') to protect extremely vulnerable patient groups deemed at high risk severe SARS-CoV-2 infection. 1These included children adults sickle cell (HbSS).On advice National Hemoglobinopathy Panel (NHP), a multidisciplinary expert advisory group, shielding guidance was extended all disease (SCD)...
The purpose of this study sickle cell disease (SCD) was to determine whether arteriopathy, measurable as intracranial vessel signal loss on magnetic resonance angiography (MRA), associated with low nocturnal hemoglobin oxygen saturation (SpO2) or hemolytic rate, reticulocytosis unconjugated hyperbilirubinemia.Ninety-five East London children SCD without prior stroke had overnight pulse oximetry, whom 47 (26 boys, 39 SS; mean age 9.1 ± 3.1 years) also MRA, transcranial Doppler (TCD),...
Summary Sickle cell disease is a multisystem characterised by chronic haemolytic anaemia, painful vaso‐occlusive crises and acute end‐organ damage. It one of the most common serious inherited single gene conditions worldwide has major impact on health affected individuals. Peri‐operative complications are higher in patients with sickle compared general population may be or non‐sickle‐related. Complications reduced meticulous peri‐operative care transfusion, but unnecessary transfusion should...
Autophagy plays an important role in the removal of membrane bound organelles during last stage erythropoiesis as enucleate reticulocyte matures into erythrocyte. Autophagic vesicles are expelled from intact, inside-out, phosphatidylserine (PS) decorated and subsequently removed splenic passage. Failure to remove these causes elevation PS exposed red cells Sickle Cell Disease.
Abstract Sickle cell disease (SCD) is one of the most common inherited single gene disorders. Polymerisation sickle hemoglobin results in erythrocytes that are inflexible and adherent, leading to coagulation, vascular cellular activation resultant blood vessel blockage. Previous studies have observed elevated numbers red cell-derived particles (RCDP), also denoted extracellular vesicles, SCD plasma. Here, imaging flow cytometry was used quantify all RCDP A more heterogenous population than...
Patients with sickle cell trait (STr) are usually considered to be asymptomatic. However, complications, including hypercoagulability, increased risk of venous thromboembolism and the exertional exercise syndrome rhabdomyolysis sudden death, have been described. The exact cause these adverse events is unclear. We investigated two patients, a set monozygotic twins STr, establish their procoagulant activity status as potential indicator thrombotic risk. In-vivo thrombin generation was assessed...
Summary Objectives To determine the organisational resources in place; what blood was being transfused, why, how, where, when and by whom; whether laboratory support policies met standards for patients with sickle cell disease (SCD). Background SCD affects 14 000 people United Kingdom (UK). Standards guidelines do not cover all aspects of transfusion there are no data on their use; may become very sick without warning presenting to non‐specialist hospitals; services increasingly supplying...
Summary Objectives To establish, in an unselected population of London haemoglobinopathy patients, transfusion requirements, blood antigens/alloantibodies, modalities, burden reactions and donor exposure. Background Haemoglobinopathy patients are among the most highly transfused patient populations, overall number on long‐term programmes increasing. provide a safe efficacious service for it is important to understand current practice, morbidity associated with transfusion, efficacy different...