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Am J Med ; 135(6): e136, 2022 Jun.
Article in English | MEDLINE | ID: covidwho-1864528
BMJ Case Rep ; 14(2)2021 Feb 18.
Article in English | MEDLINE | ID: covidwho-1186243


A 53-year-old man presented to a district general hospital with chest pain, ECG changes and a small high-sensitivity cardiac troponin I rise. There were no symptoms of heart failure. CT coronary angiography revealed moderate calcific disease and conventional angiography confirmed no flow limitation. Echocardiography showed left ventricular hypertrophy (LVH). His blood pressure remained normal throughout his admission. The tertiary centre labelled this as a 'plaque rupture' event but the LVH remained unexplained. Cardiac MRI displayed an unusual pattern of late gadolinium enhancement, which was not classical of amyloid. However, a raised serum free kappa light chain along with the deposition of amyloid on his bone marrow aspirate confirmed the diagnosis of primary AL amyloidosis with cardiac involvement. The patient went on to have chemotherapy and remained stable at 1-year follow-up.

Acute Coronary Syndrome , Amyloidosis , Heart Failure , Acute Coronary Syndrome/diagnosis , Amyloidosis/diagnosis , Contrast Media , Gadolinium , Humans , Male , Middle Aged
Amyloid ; 27(4): 217-222, 2020 Dec.
Article in English | MEDLINE | ID: covidwho-889371


The ISA Nomenclature Committee met electronically before and directly after the XVII ISA International Symposium on Amyloidosis, which, unfortunately, had to be virtual in September 2020 due to the ongoing COVID-19 pandemic instead of a planned meeting in Tarragona in March. In addition to confirmation of basic nomenclature, several additional concepts were discussed, which are used in scientific amyloid literature. Among such concepts are cytotoxic oligomers, protofibrils, primary and secondary nucleation, seeding and cross-seeding, amyloid signature proteins, and amyloid plaques. Recommendations for their use are given. Definitions of amyloid and amyloidosis are confirmed. Possible novel human amyloid fibril proteins, appearing as 'classical' in vivo amyloid, were discussed. It was decided to include fibulin-like extracellular matrix protein 1 (amyloid protein: AEFEMP1), which appears as localised amyloid in portal veins. There are several possible amyloid proteins under investigation, and these are included in a new Table.

Amyloid/classification , Amyloidogenic Proteins/classification , Amyloidosis/classification , Terminology as Topic , Amyloid/genetics , Amyloid/metabolism , Amyloidogenic Proteins/genetics , Amyloidogenic Proteins/metabolism , Amyloidosis/diagnosis , Amyloidosis/genetics , Amyloidosis/pathology , COVID-19 , Congresses as Topic , Coronavirus Infections , Education, Distance/organization & administration , Gene Expression , Humans , Pandemics , Pneumonia, Viral