J Magn Reson Imaging 2021 Jul 23. Epub 2021 Jul 23.
Department of Bioengineering, The University of Texas at Dallas, Richardson, Texas, USA.
Amyloid 2021 Sep 22:1-7. Epub 2021 Sep 22.
Department of Clinical Therapeutics, National Kapodistrian University of Athens, Medical School, Athens, Greece.
Background: The utilization and clinical impact of beta-blockers (BBs) in cardiac amyloidosis (CA) is largely unexplored.Methods: We conducted a retrospective, single-center analysis of indications, timing of initiation, types and doses of BB used, reasons to discontinue BB and association between BB tolerance and outcomes in a cohort of patients with immunoglobulin light chain amyloidosis (AL).Results: We reviewed 236 patients with AL CA and identified 53 patients taking BB (22. Read More
BMC Cardiovasc Disord 2021 Sep 18;21(1):452. Epub 2021 Sep 18.
Department of Cardiovascular Medicine, The Second Xiangya Hospital of Central South University, No. 139 Middle Renmin Road, Furong District, Changsha City, 410011, Hunan Province, China.
Background: Light-chain cardiac amyloidosis (AL-CA) has been highly valued in developed countries, but in developing countries, the recognition and diagnosis of this condition is still limited. There are currently few reports on a large number of Chinese patients with AL-CA. The present study aimed to report real-world clinical characteristics and prognosis of AL-CA in China. Read More
Ann Transl Med 2021 Aug;9(16):1347
Department of Hematology, Shengjing Hospital of China Medical University, Shenyang, China.
Background: Light-chain (AL) amyloidosis frequently involves severe multiple end-organ damage, thus affecting prognosis. As the current disease staging system is based only on cardiac indicators, we propose a new staging system based on multiple organ indicators to supplement the existing system.Methods: Patients with AL amyloidosis (n=1,064) from 18 Chinese hospitals were enrolled and divided into test and validation cohorts (4:1). Read More
Neurol Sci 2021 Sep 16. Epub 2021 Sep 16.
Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, 466-8550, Japan.
Paraproteinemia is associated with different peripheral neuropathies. The major causes of neuropathy correlated with paraproteinemia are the deposition of immunoglobulin in the myelin, represented by anti-myelin-associated glycoprotein (MAG) neuropathy; deposition of immunoglobulin or its fragment in the interstitium, represented by immunoglobulin light chain amyloidosis (AL amyloidosis); and paraneoplastic mechanisms that cannot be solely attributed to the deposition of immunoglobulin or its fragment, represented by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin change (POEMS) syndrome. Patients with anti-MAG neuropathy and POEMS syndrome present with slowing of nerve conduction parameters. Read More
ESC Heart Fail 2021 Sep 16. Epub 2021 Sep 16.
Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-Ro Gangnam-gu, Seoul, 06351, Republic of Korea.
Aims: Cardiac involvement is crucial factor determining outcomes of light chain (AL) amyloidosis. This study evaluated whether capillary density (CD) quantified from endomyocardial biopsy is associated with structural and functional parameters of amyloid heart. Also, we investigated whether capillary density improves the prognostic value of the current staging system in AL amyloidosis patients with cardiac involvement. Read More