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      Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis.

      1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1 , 1
      Circulation
      Ovid Technologies (Wolters Kluwer Health)
      amyloid, cardiomyopathies, genetics, hypertrophy, radionuclide imaging
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopathy for which several promising therapies are in development. The diagnosis is frequently delayed or missed because of the limited specificity of echocardiography and the traditional requirement for histological confirmation. It has long been recognized that technetium-labeled bone scintigraphy tracers can localize to myocardial amyloid deposits, and use of this imaging modality for the diagnosis of cardiac ATTR amyloidosis has lately been revisited. We conducted a multicenter study to ascertain the diagnostic value of bone scintigraphy in this disease.

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          Most cited references4

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          Highly sensitive, automated immunoassay for immunoglobulin free light chains in serum and urine.

          Bence Jones proteins or monoclonal immunoglobulin kappa and lambda free light chains (FLCs) are important markers for identifying and monitoring many patients with B-cell tumors. Automated immunoassays that measure FLCs in urine and serum have considerable clinical potential. Sheep antibodies, specific for FLCs, were prepared by immunization with pure kappa and lambda molecules and then adsorbed extensively against whole immunoglobulins. The antibodies were conjugated onto latex particles and used to assay kappa and lambda FLCs on the Beckman IMMAGE protein analyzer. The unconjugated antibodies showed minimal cross-reactivity with intact immunoglobulins or other proteins. With latex-conjugated antibodies, kappa and lambda FLCs could be measured in normal sera and most normal urine samples. Patients with multiple myeloma had increased concentrations of the relevant serum FLC, whereas both FLCs were increased in the sera of patients with systemic lupus erythematosus. We developed sensitive, automated immunoassays for kappa and lambda FLC measurements in serum and urine that should facilitate the assessment of patients with light chain abnormalities.
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            Primary sistemic amyloidosis: clinical and laboratory features in 474 cases

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              V122I transthyretin variant in elderly black Americans.

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                Author and article information

                Journal
                Circulation
                Circulation
                Ovid Technologies (Wolters Kluwer Health)
                1524-4539
                0009-7322
                Jun 14 2016
                : 133
                : 24
                Affiliations
                [1 ] From National Amyloidosis Centre, Division of Medicine (J.D.G., A.D.W., C.C.Q., H.J.L., T.R., M.F., K.F., T.L., F.W.V., C.J.W., D.F.H., P.N.H.) and Barts Heart Centre, Institute of Cardiovascular Science (J.C.M.), University College London, UK; Clinical Cardiovascular Research Laboratory for the Elderly, Columbia University Medical Center, New York (M.S.M., S.B., A.C.); Department of Cardiology and Department of Radiology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA (R.H.F., S.D.); Amyloidosis Research and Treatment Center, Foundation Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, and Department of Molecular Medicine, University of Pavia, Italy (G.M., G.P., P.M.); Department of Cardiology, University Hospital Henri Mondor, Amyloidosis Mondor Network, Creteil, France (T.D.); Division of Hematology, Division of Cardiovascular Diseases, and Department of Radiology, Division of Nuclear Medicine, Department of Medicine, Mayo Clinic, Rochester, MN (A.D., M.G., G.B.J.); Amyloidosis Center, Boston University School of Medicine and Boston Medical Center, MA (J.L.B., F.L.R.); Departments of Nuclear Medicine and Molecular Imaging (A.W.J.M.G.) and Rheumatology and Clinical Immunology (B.P.H.), University of Groningen, University Medical Center Groningen, The Netherlands; Department of Nuclear Medicine, S. Orsola-Malpighi Hospital, and Cardiology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater-University of Bologna, Italy (P.L.G., C.R.); and Yale University School of Medicine, Section of Cardiovascular Medicine, New Haven, CT (E.J.M.).
                Article
                CIRCULATIONAHA.116.021612
                10.1161/CIRCULATIONAHA.116.021612
                27143678
                3a11aa49-33dd-4596-b0b1-4351a32215c9
                History

                amyloid,cardiomyopathies,genetics,hypertrophy,radionuclide imaging

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