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POEMS Syndrome.
Presse Med
January 2025
Department of Hematology and Cellular Therapy, National Reference Center "AL Amyloidosis and Other Monoclonal Immunoglobulin Deposit Diseases, University Hospital of Limoges, Limoges, France.
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, Skin changes) is a syndrome that involves a monoclonal B-cell proliferation, most often plasmacytic, and a variable number of manifestations listed or not in the acronym. These manifestations include sclerotic bone lesions, plasmacytic Castleman disease, papillary edema, peripheral edema, ascites, thrombocytosis and/or polycythemia, venous and/or arterial thrombosis, and renal, pulmonary, and cardiac impairments . Diagnosis is often delayed due to the rarity of this entity and its clinical polymorphism, which can mimic other neurological disorders.
View Article and Find Full Text PDFThe Accuracy of Technetium-99 m Pyrophosphate Imaging in Diagnosing Transthyretin Cardiac Amyloidosis and Its Impact on Patient Management.
Curr Cardiol Rep
January 2025
Department of Cardiac Sciences, University of Calgary and Libin Cardiovascular Institute, Calgary, AB, Canada.
Purpose Of Review: This review evaluates recent advancements in Technetium-99 m pyrophosphate (99mTc-PYP) imaging for transthyretin amyloid cardiomyopathy (ATTR-CM). We summarize the advantages of single-photon emission computed tomography (SPECT) over planar imaging, the potential impact of quantitative methods, and emerging data for quantifying response to therapy.
Recent Findings: The current literature demonstrates the superior diagnostic accuracy of SPECT compared with planar imaging in 99mTc-PYP studies.
Can Outpatient Worsening Heart Failure Serve as an Endpoint in Clinical Trials of Transthyretin Amyloidosis?
J Am Coll Cardiol
January 2025
Section of Cardiovascular Medicine, Boston University Chobanian & Avedisian School of Medicine and Boston Medical Center, Boston, Massachusetts, USA. Electronic address:
Worsening of Heart Failure in Outpatients With Transthyretin Amyloidosis and Cardiomyopathy in the APOLLO-B Trial.
J Am Coll Cardiol
December 2024
Cardiovascular Division, Brigham and Women's Hospital, Boston, Massachusetts, USA. Electronic address:
Background: Outpatient worsening heart failure (HF), defined by initiation or intensification of diuretics, is adversely prognostic for patients with either reduced or preserved ejection fraction.
Objectives: This study sought to investigate the prognostic value of outpatient worsening HF in transthyretin amyloidosis with cardiomyopathy and the effect of patisiran treatment.
Methods: Post hoc analyses of the APOLLO-B trial (NCT03997383) evaluated the associations between outpatient worsening HF (defined by oral diuretic initiation or intensification), measures of disease progression, and a composite endpoint of all-cause mortality and cardiovascular (CV) events.
Advances in genetic diagnosis and therapy of hereditary heart disease: a bibliometric review from 2004 to 2024.
Front Med (Lausanne)
January 2025
Key Laboratory of Obstetric and Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, Department of Radiology, West China Second University Hospital, Sichuan University, Chengdu, China.
Hereditary heart disease (HHD) is a series of cardiac disorders associated with monogenic or polygenic abnormalities and is one of the leading causes of sudden death, particularly in young adults. The updated European Cardiology guideline for cardiomyopathies provides the first comprehensive summary of genotyping, imaging, and therapy recommendations for inherited cardiomyopathies, but still lacks a comprehensive discussion of research advances and future trends in genetic diagnosis and therapy of HHD. Our research aims to fill this gap.
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