A5081630725- Amyloidosis: Diagnosis, Treatment, Outcomes
- Parathyroid Disorders and Treatments
- Medical Imaging and Pathology Studies
- Cardiomyopathy and Myosin Studies
- Eosinophilic Disorders and Syndromes
- Dermatological and Skeletal Disorders
- Sarcoidosis and Beryllium Toxicity Research
- Trace Elements in Health
- Peptidase Inhibition and Analysis
- Pneumocystis jirovecii pneumonia detection and treatment
- Pancreatitis Pathology and Treatment
- Neuroendocrine Tumor Research Advances
- IgG4-Related and Inflammatory Diseases
- Connective tissue disorders research
- Caveolin-1 and cellular processes
- Bone health and treatments
- Lanthanide and Transition Metal Complexes
- Hepatitis C virus research
- Renal Diseases and Glomerulopathies
- Radiopharmaceutical Chemistry and Applications
- Liver Disease Diagnosis and Treatment
- Cardiovascular Function and Risk Factors
- Monoclonal and Polyclonal Antibodies Research
- MRI in cancer diagnosis
- Clostridium difficile and Clostridium perfringens research
University College London
2015-2024
Amyloidosis Foundation
2017-2024
The Royal Free Hospital
2014-2023
Royal Free London NHS Foundation Trust
2022
Children's Cancer and Leukaemia Group
2016-2022
University College Hospital
2020
National Health Service
2018
Great Ormond Street Hospital
2016
St Bartholomew's Hospital
2016
Columbia University Irving Medical Center
2016
Background— Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopathy for which several promising therapies are in development. The diagnosis frequently delayed or missed because of the limited specificity echocardiography traditional requirement histological confirmation. It has long been recognized that technetium-labeled bone scintigraphy tracers can localize to myocardial amyloid deposits, use this imaging modality cardiac ATTR lately revisited. We conducted...
Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure in older individuals. We sought to characterize the natural history ATTR-CM and compare outcomes quality life among patients with acquired hereditary forms disease.We studied 711 wild-type ATTR-CM, 205 associated V1221 variant (V122I-hATTR-CM), 118 non-V122I-hATTR-CM at UK National Amyloidosis Center between 2000 2017. Patients underwent prospective protocolized evaluations comprising...
Background— Calcific aortic stenosis (cAS) affects 3% of individuals aged >75 years, leading to heart failure and death unless the valve is replaced. Wild-type transthyretin cardiac amyloid also a disorder ageing individuals. Prevalence clinical significance dual pathology are unknown. This study explored prevalence wild-type in cAS by myocardial biopsy, its imaging phenotype prognostic significance. Methods Results— A total 146 patients with severe AS requiring surgical replacement...
Technetium-99m-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid ((99m)Tc-DPD) is a sensitive method for imaging cardiac transthyretin (ATTR) amyloid. We report utility and limitations of (99m)Tc-DPD scintigraphy in 321 patients with suspected amyloidosis.The cohort included wild-type ATTR (ATTRwt) amyloidosis 94 (29%), ATTR-Val122Ile 38 (12%), hereditary (ATTRmt) 46 (14%), primary light-chain (AL) 44 secondary (AA) three (1%), other types nine (3%), undetermined two (0.5%), 85 (26.5%)...
Purpose To investigate cardiac magnetic resonance (MR) imaging measurements of extracellular volume (ECV) and total cell in immunoglobulin light-chain amyloidosis (AL) transthyretin (ATTR) order to evaluate the amyloid myocyte volumes. Materials Methods All ethics were approved, participants provided written informed consent. Of 257 subjects who recruited, 92 had AL (mean age, 62 years ± 10), 44 mutant ATTR 68 66 wild-type 75 7). In addition, eight healthy with mutations 47 6) volunteers 45...
Transthyretin amyloid cardiomyopathy (ATTR-CM) is often assumed to be associated with wild-type TTR genotype (ATTRwt) in elderly patients (aged ≥70), some of whom are not offered genetic testing. We sought estimate the prevalence, clinical characteristics and prognostic implications transthyretin (TTR) variants among diagnosed ATTR-CM.Data from consecutive over 70 years age ATTR-CM at UK National Amyloidosis Centre between January 2010 August 2022 were retrospectively evaluated. All...
Abstract Aims To perform evaluation of widely embraced bone scintigraphy-based non-biopsy diagnostic criteria (NBDC) for ATTR amyloid cardiomyopathy (ATTR-CM) in clinical practice, and to refine serum free light chain (sFLC) ratio cut-offs that reliably exclude monoclonal gammopathy (MG) chronic kidney disease. Methods results A multi-national retrospective study 3354 patients with suspected or histologically proven cardiac amyloidosis (CA) referred specialist centres from 2015 2021;...
BACKGROUND: The extent of myocardial bone tracer uptake with technetium pyrophosphate, hydroxymethylene diphosphonate, and 3,3-diphosphono-1,2-propanodicarboxylate in transthyretin amyloid cardiomyopathy (ATTR-CM) might reflect cardiac burden be associated outcome. METHODS: Consecutive patients ATTR-CM who underwent diagnostic scintigraphy acquisition whole-body planar single-photon emission computed tomography (SPECT) images from the National Amyloidosis Centre 4 Italian centers were...
High-grade (Perugini grade 2 or 3) cardiac uptake on bone scintigraphy with 99mTechnetium labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) has lately been confirmed to have high diagnostic sensitivity and specificity for transthyretin (ATTR) amyloidosis. We sought determine whether patient stratification by Perugini 99mTc-DPD prognostic significance in ATTR amyloidosis.Patient survival from time of was determined 602 patients amyloidosis, including 377 wild-type (ATTRwt) 225...
Abstract Aims Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure. We sought to characterize the structural and functional echocardiographic phenotype across spectrum wild-type (wtATTR-CM) hereditary (hATTR-CM) transthyretin features predicting prognosis. Methods results studied 1240 patients with ATTR-CM who underwent prospective protocolized evaluations comprising full assessment survival between 2000 2019, 766 wtATTR-CM 474 hATTR-CM,...
Repeat cycles of miridesap, to deplete circulating serum amyloid P component (SAP), followed by the anti-SAP antibody, dezamizumab, cleared visceral deposits in patients with systemic amyloidosis.
Transthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly diagnosed at an early stage of the disease natural history, defined as National Amyloidosis Centre (NAC) ATTR Stage I. The history early-stage ATTR-CM remains poorly characterized.
Objective: Systemic AA amyloidosis is a serious complication of chronic inflammation; however, there are relatively few published data on its incidence. We investigated the changing epidemiology over 25-year period at single national referral centre.Methods: conducted retrospective study all patients diagnosed with who had attended centre between 1990 and 2014 inclusive. Six hundred twenty-five were studied in three cohorts: C1: 1990–1997; C2: 1998–2006; C3: 2007–2014.Results: Mean age...
Technetium-99m-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD scintigraphy) is recognized as highly accurate for the non-invasive diagnosis of transthyretin (ATTR) cardiac amyloidosis (CA). A proportion patients with immunoglobulin light chain (AL) CA have also been reported to show 99mTc-DPD uptake. Herein, we assessed frequency and degree uptake its clinical significance among AL CA.Between 2010 2017, 292 consecutive underwent scintigraphy were included in this study: 114...
Systemic light chain amyloidosis is a multisystem disorder that commonly involves the heart, liver, and spleen. Cardiac magnetic resonance with extracellular volume (ECV) mapping provides surrogate measure of myocardial, spleen amyloid burden. The purpose this study was to assess multiorgan response treatment using ECV mapping, association between prognosis. authors identified 351 patients who underwent baseline serum amyloid-P-component (SAP) scintigraphy cardiac at diagnosis, which 171 had...
Transthyretin (ATTR) amyloidosis is a rare but serious infiltrative disease associated with wide spectrum of morphologic and functional cardiac involvement. 99mTc-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (DPD), initially developed as bone-seeking radiotracer, remarkably sensitive for imaging ATTR amyloid deposits. Our aim was to investigate the feasibility utility estimating 99mTc-DPD uptake in myocardial tissue; this has potential yield reliable quantitative information on...
Aims Transthyretin cardiac amyloidosis (ATTR-CM) is a progressive and fatal cardiomyopathy. Treatment options in patients with advanced ATTR-CM are limited to transplantation (CT). Despite case series demonstrating comparable outcomes CT between non-amyloid cardiomyopathies, considered be contraindication some centers, partly due perceived risk of amyloid recurrence the allograft. We report long-term at two tertiary centers. Materials methods Results retrospectively evaluated across centers...
Abstract Aims Transthyretin amyloid cardiomyopathy (ATTR‐CM) is an increasingly recognized cause of heart failure. A total 3–4% individuals African descent carry a TTR gene mutation encoding the p.(V142I) variant, powerful risk factor for development variant ATTR‐CM (ATTRv‐CM); this equates to 1.6 million carriers in United States. We undertook deep phenotyping p.(V142I)‐ATTRv‐CM and comparison with wild‐type (ATTRwt‐CM). Methods results retrospective study 413 patients ATTRv‐CM who attended...
Cardiac transthyretin amyloidosis (ATTR-CM) is a progressive and fatal condition. Prognosis can be determined at diagnosis according to the National Amyloidosis Centre (NAC) (ATTR) stage. We sought examine how NAC ATTR stage changes during follow-up whether it maintains its prognostic value throughout disease course.We performed retrospective study of 945 patients with wild-type ATTR-CM (wtATTR-CM) or hereditary associated V122I variant (V122I-hATTR-CM) who were diagnosed serially evaluated...
Systemic amyloidosis is characterized by amyloid deposition that can involve virtually any organ. Splenic and hepatic occurs in certain types, some patients but not others, may influence prognosis treatment. SAP (serum P component) scintigraphy uniquely able to identify quantify the liver spleen, thus informing clinical management, it only available 2 centers globally. The aims of this study were examine potential for extracellular volume (ECV) mapping performed during routine cardiac...