Advances in the treatment of hereditary transthyretin amyloidosis: A review
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DOI: 10.1002/brb3.1371
Abstract: Amyloid transthyretin amyloidosis (ATTR) is a progressive and often fatal disease caused by the buildup of mutated (hereditary ATTR [hATTR]; also known as ATTR variant [ATTRv]) or normal transthyretin (wild‐type ATTR) throughout the body. Two… read more here.
Keywords: transthyretin amyloidosis; amyloidosis; advances treatment; attr ... See more keywords
Efficacy and safety of tafamidis doses in the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR‐ACT) and long‐term extension study
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DOI: 10.1002/ejhf.2027
Abstract: Tafamidis is an effective treatment for transthyretin amyloid cardiomyopathy (ATTR‐CM) in the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR‐ACT). While ATTR‐ACT was not designed for a dose‐specific assessment, further analysis from ATTR‐ACT and its long‐term… read more here.
Keywords: attr act; attr; tafamidis transthyretin; transthyretin cardiomyopathy ... See more keywords
Use of fluorine-18 sodium fluoride for the diagnosis of transthyretin cardiac amyloidosis: The birth of a new technique
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DOI: 10.1007/s12350-017-0847-6
Abstract: Imaging techniques undergo a process of development. They are birthed from an idea or a hypothesis, and then investigators look for pre-clinical models followed by publication of clinical case reports. In the adolescence of a… read more here.
Keywords: technique; medicine; cardiology; amyloidosis ... See more keywords
Optimal interpretation of Tc99m PYP in 2020: Avoiding the million-dollar mistake
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DOI: 10.1007/s12350-020-02503-8
Abstract: The diagnosis of transthyretin cardiac amyloidosis (ATTR-CA) is now literally a million-dollar implication. With the approval of tafamidis (Vyndaqel/ Vyndamax) in 2019, ATTR-CA has become a treatable chronic disease state with potentially life prolonging therapy… read more here.
Keywords: interpretation; million dollar; cardiology; pyp imaging ... See more keywords
Evaluating Use of 99mTc-PYP Scan in Diagnosis of ATTR- A Tertiary Care Center Experience
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DOI: 10.1016/j.healun.2021.01.617
Abstract: Purpose The 99mTc-pyrophosphate (PYP) scan for the diagnosis of Transthyretin Related Cardiac Amyloidosis (ATTR) may be used in an inappropriate context or with incorrect interpretation, leading to misdiagnosis and futile financial burden. Here we report… read more here.
Keywords: scan diagnosis; attr; use; pyp scan ... See more keywords
Impact of Earlier Diagnosis in Cardiac ATTR Amyloidosis Over the Course of 20 Years
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DOI: 10.1161/circulationaha.122.060852
Abstract: Background: Diagnostic and therapeutic advances have led to much greater awareness of transthyretin cardiac amyloidosis (ATTR-CA). We aimed to characterize changes in the clinical phenotype of patients diagnosed with ATTR-CA over the past 20 years.… read more here.
Keywords: attr; diagnosis; versus versus; patients referred ... See more keywords
Patient and family experience with transthyretin amyloid cardiomyopathy (ATTR-CM) and polyneuropathy (ATTR-PN) amyloidosis: results of two focus groups
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DOI: 10.1186/s13023-021-01706-7
Abstract: Background Transthyretin amyloidosis, or ATTR, is a progressive and debilitating rare proteopathy generally manifested as either transthyretin amyloid polyneuropathy (ATTR-PN) or transthyretin amyloid cardiomyopathy (ATTR-CM). Irrespective of the clinical presentation, affected patients manage a chronic… read more here.
Keywords: polyneuropathy attr; family; transthyretin amyloid; attr ... See more keywords
Late-onset Hereditary ATTR Amyloidosis with a Novel p.P63S (P43S) Transthyretin Variant
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DOI: 10.2169/internalmedicine.5615-20
Abstract: The patient was an 82-year-old Japanese man with no family history suggestive of amyloidosis. He developed bilateral leg edema and shortness of breath and was referred to our hospital. An electrocardiogram showed atrial fibrillation with… read more here.
Keywords: variant; amyloidosis; attr; p63s p43s ... See more keywords
How did transthyretin amyloid cardiomyopathy progress in patients who took placebo in the study ATTR-ACT? A plain language summary.
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DOI: 10.2217/fca-2021-0150
Abstract: WHAT IS THIS PLAIN LANGUAGE SUMMARY ABOUT? This plain language summary describes some results of a study called ATTR-ACT. This was the first large study to include people with wild-type and hereditary transthyretin amyloid cardiomyopathy… read more here.
Keywords: attr; took placebo; placebo; heart ... See more keywords
Towards a Diagnosis of Cardiac Amyloidosis: Single Center Experience with 99m Technetium Pyrophosphate Planar Imaging and Opportunities for Standardization of Diagnostic Workflow
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DOI: 10.3390/medicina59020378
Abstract: Background and Objectives: Cardiac amyloidosis is a disorder caused by amyloid fibril deposition in the extracellular space of the heart. Almost all forms of clinical cardiac amyloidosis are transthyretin amyloidosis (ATTR) or light chain amyloidosis.… read more here.
Keywords: cardiac amyloidosis; attr; diagnosis; workflow ... See more keywords
Spectrum of transthyretin gene mutations and clinical characteristics of Polish patients with cardiac transthyretin amyloidosis.
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DOI: 10.5603/cj.a2020.0104
Abstract: BACKGROUND Transthyretin amyloidosis (ATTR) is a rare, life-threatening systemic disorder. We present first findings on the cardiac hereditary ATTR in Poland. METHODS Sixty-eight consecutive patients with suspected or known cardiac amyloidosis were evaluated, including blood… read more here.
Keywords: transthyretin amyloidosis; attr; amyloidosis; gene mutations ... See more keywords