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Methods Fifty patients with cATTR who underwent both planar whole-body DPD scintigraphy and nerve conduction studies were retrospectively evaluated, according to Fifty patients with cATTR. Patients with cATTR and additional diagnosis of PNP were significantly higher than those without compared to patients without. When compared to patients without, Quantitative SPECT/CT reported that DPD in the subcutaneous fat of the left axillar region was markedly elevated in cATTR patients. Conclusions DPD bone scintigraphy is a useful tool for identifying patients with cATTR and a risk for PNP, according to the increased DPD soft tissue uptake.
Source link: https://europepmc.org/article/MED/35817943
ATTR amyloidosis is a fatal disease linked to the buildup of transthyretin fibrils, leading to organ failure and death. Although ATTR amyloidosis patients accumulate ATTR fibrils in virtually every organ, their clinical appearance is often unpredictable and variable. Both patients of the same disease, tau and u03b1-synucleinopathies, diseases involving amyloidosis of tau and u03b1-synuclein, and u03b1-synuclein, have the same fibril fold, or polymorph, according to recent studies in cryoelectron microscopy. We use cryo-EM to see if fibrils from heart tissue of various patients with cardiac ATTR amyloidosis have a common fold in this research. We found that ATTRv mutations alter the fibril structure, which is why ATTRwt fibrils have a common fold. Our results show that unlike in tauopathies and synucleinopathies, ATTRv fibrils exhibit structural polymorphism triggered by each individual and their genotypes.
Source link: https://europepmc.org/article/PPR/PPR508502
Using the Perugini scoring system and the heart/contralateral heart ratio on planar imaging, scintigraphy provides qualitative and semi-quantitative diagnosis of ATTR cardiovascular amyloidosis. We investigated the relationship between xSPECT/CT SUVs and Perugini's score and H/CL ratio. xSPECT/CT reports a retrospective review of 78 patients with suspected ATTR-CA who underwent 99mTc-PYP scintigraphy with xSPECT/CT. [SUV max myocardium/SUV max vertebrae] u00d7 SUV max spinal muscle was calculated as [SUV max myocardium/SUV max vertebrae] adequate muscle was measured in Cardiac's retention index, which was calculated as [SUV max myocardium/SUV max vertebrae] ad7 SUV max maximum spine muscle, u00d7 SUV max spine muscle, ad7 SUV max of the U00d7 SUV max vertebrae Myocardium SUV max at 1-hour correlation with Perugini grades, H/CL ratio, CMR, and echocardiographic results were all present at one hour in a 1-hour correlation with Perugini grades, H/CL ratio, CMR, and echocardiographic results. The difference between Perugini Grade 3 and 1 corresponded to higher myocardium SUV max values, particularly when comparing Perugini Grade 3 to Grade 2 and 1. In addition, patients with H/CL u2265 1. 5 had a significant myocardium SUV max compared to patients with H/CL u2264 1. 5. The Myocardium SUV max at 1-hour was highly correlated with ECV, pre-contrast T1 map values, and left ventricle mass index on CMR.
Source link: https://europepmc.org/article/MED/35655113
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