Results 1 to 10 of about 200,281 (336)

Magnetization transfer ratio quantifies polyneuropathy in hereditary transthyretin amyloidosis

Annals of Clinical and Translational Neurology, 2020
Objective To quantify peripheral nerve lesions in symptomatic and asymptomatic hereditary transthyretin amyloidosis with polyneuropathy (ATTRv‐PNP) by analyzing the magnetization transfer ratio (MTR) of the sciatic nerve, and to test its potential as a ...
Jennifer Kollmer, Ute Hegenbart, Christoph Kimmich, Ernst Hund, Jan C. Purrucker, John M. Hayes, Stephen I. Lentz, Georges Sam, Johann M. E. Jende, Stefan O. Schönland, Martin Bendszus, Sabine Heiland, Markus Weiler   +12 more
doaj   +2 more sources

Combined Subcutaneous Fat Aspirate and Skin Tru-Cut Biopsy for Amyloid Screening in Patients with Suspected Systemic Amyloidosis

Molecules, 2021
Screening for systemic amyloidosis is typically carried out with abdominal fat aspirates with varying reported sensitivities. Fat aspirates are preferred for use in primary screening instead of organ biopsies as they are less invasive and thereby ...
Charlotte Toftmann Hansen, Hanne E. H. Møller, Aleksandra Maria Rojek, Niels Marcussen, Hans Christian Beck, Niels Abildgaard   +5 more
doaj   +1 more source

Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib

Blood Cancer Journal, 2021
The outcomes in systemic AL amyloidosis are dependent on the depth of haematologic response. However, there is limited data on the impact of the speed of response on outcomes.
Sriram Ravichandran, Oliver C. Cohen, Steven Law, Darren Foard, Marianna Fontana, Ana Martinez-Naharro, Carol Whelan, Julian D. Gillmore, Helen J. Lachmann, Sajitha Sachchithanantham, Shameem Mahmood, Philip N. Hawkins, Ashutosh D. Wechalekar   +12 more
doaj   +1 more source

Cardiac biomarkers are prognostic in systemic light chain amyloidosis with no cardiac involvement by standard criteria

Haematologica, 2020
Patients with systemic immunoglobulin light chain amyloidosis (AL) with no evidence of cardiac involvement by consensus criteria have excellent survival, but 20% will die within 5 years of diagnosis and prognostic factors remain poorly characterised.
Faye A. Sharpley, Marianna Fontana, Ana Martinez-Naharro, Richa Manwani, Shameem Mahmood, Sajitha Sachchithanantham, Helen J. Lachmann, Julian D. Gillmore, Carol J. Whelan, Philip N. Hawkins, Ashutosh D. Wechalekar   +10 more
doaj   +1 more source

First third-generation CAR T cell application targeting CD19 for the treatment of systemic IgM AL amyloidosis with underlying marginal zone lymphoma

Biomarker Research, 2023
Light chain amyloidosis (AL) is a rare disease caused by the generalized deposition of misfolded free light chains. Patients with immunoglobulin M gammopathy (IgM) and indolent B-cell lymphoma such as marginal zone lymphoma (MZL) may in some instances ...
Felix Korell, Stefan Schönland, Anita Schmitt, Madelaine Jansen, Kiavasch Farid, Carsten Müller-Tidow, Peter Dreger, Michael Schmitt, Ute Hegenbart   +8 more
doaj   +1 more source

Paraskeletal and extramedullary plasmacytomas in multiple myeloma at diagnosis and at first relapse: 50-years of experience from an academic institution

Blood Cancer Journal, 2022
From January 1970 to December 2018, 1304 patients were diagnosed with multiple myeloma (MM) at our institution and 256 (19.6%) had plasmacytomas (Ps) (paraskeletal –PPs- 17.6%, extramedullary –EMPs-1.9%).
Raquel Jiménez-Segura, Laura Rosiñol, Ma Teresa Cibeira, Carlos Fernández de Larrea, Natalia Tovar, Luis Gerardo Rodríguez-Lobato, Esther Bladé, David F. Moreno, Aina Oliver-Caldés, Joan Bladé   +9 more
doaj   +1 more source

Arrhythmic Burden in Cardiac Amyloidosis: What We Know and What We Do Not

Biomedicines, 2022
Cardiac amyloidosis (CA), caused by the deposition of insoluble amyloid fibrils, impairs different cardiac structures, altering not only left ventricle (LV) systo-diastolic function but also atrial function and the conduction system.
Alessia Argirò, Annamaria Del Franco, Carlotta Mazzoni, Marco Allinovi, Alessia Tomberli, Roberto Tarquini, Carlo Di Mario, Federico Perfetto, Francesco Cappelli, Mattia Zampieri   +9 more
doaj   +1 more source

Health-related quality of life in hereditary transthyretin amyloidosis polyneuropathy: a prospective, observational study [PDF]

, 2020
Background Hereditary Transthyretin Amyloidosis Polyneuropathy is a rare life-threatening neurologic disease that imposes considerable mortality and it is associated with progressive related disabilities.
Coelho, Teresa, Conceição, Isabel, Costa, João, de Carvalho, Mamede, Ferreira, Lara, Inês, Mónica   +5 more
core   +2 more sources

Disease progression in cardiac transthyretin amyloidosis is indicated by serial calculation of National Amyloidosis Centre transthyretin amyloidosis stage

ESC Heart Failure, 2020
Aims Cardiac transthyretin amyloidosis (ATTR‐CM) is a progressive and fatal condition. Prognosis can be determined at diagnosis according to the National Amyloidosis Centre (NAC) transthyretin amyloidosis (ATTR) stage.
Steven Law, Aviva Petrie, Liza Chacko, Oliver C. Cohen, Sriram Ravichandran, Janet A. Gilbertson, Dorota Rowczenio, Ashutosh Wechalekar, Ana Martinez‐Naharro, Helen J. Lachmann, Carol J. Whelan, David F. Hutt, Philip N. Hawkins, Marianna Fontana, Julian D. Gillmore   +14 more
doaj   +1 more source

Plasmin activity promotes amyloid deposition in a transgenic model of human transthyretin amyloidosis

Nature Communications, 2021
ATTR amyloidosis causes heart failure through the accumulation of misfolded transthyretin in cardiac muscle. Here the authors report a mouse model of ATTR amyloidosis and demonstrate the involvement of protease activity in ATTR amyloid deposition.
Ivana Slamova, Rozita Adib, Stephan Ellmerich, Michal R. Golos, Janet A. Gilbertson, Nicola Botcher, Diana Canetti, Graham W. Taylor, Nigel Rendell, Glenys A. Tennent, Guglielmo Verona, Riccardo Porcari, P. Patrizia Mangione, Julian D. Gillmore, Mark B. Pepys, Vittorio Bellotti, Philip N. Hawkins, Raya Al-Shawi, J. Paul Simons   +18 more
doaj   +1 more source