منابع مشابه
Imaging cardiac ATTR amyloid
The heart is the principal site of involvement in nonhereditary ATTR amyloidosis and a major driver of treatment options and prognosis in hereditary forms of the disease. The goals of cardiac imaging in amyloidosis are to aid diagnosis, provide prognostic information, track disease progression and evaluate response to therapy. Echocardiography has long been the mainstay of cardiac evaluation in...
متن کاملAL and ATTR cardiac amyloid are different: native T1 mapping and ECV detect different biology
Background Cardiac involvement drives prognosis in amyloidosis. ECV directly quantitates interstitial expansion and hence myocardial amyloid burden. Native T1 is also elevated in amyloid but reflects both cell and interstitial changes. The combination gives insight into amyloid burden and the myocyte response. Previously we have shown the utility of both techniques in AL amyloidosis. Here, we e...
متن کاملOphtalmologic changes in transthyretin familial amyloid polyneuropathy (ATTR-FAP)
Background Familial amyloid polyneuropathy (FAP) is an inherited disorder with autosomal dominant transmission and multiple phenotypes, characterized by systemic accumulation of amyloid fibrils. The most common type of FAP is related to a mutant transthyretin (TTR). TTR is mainly synthesized in the liver, but few amount of TTR is produced in the eye, namely in retinal pigment epithelium, which ...
متن کاملATTR Amyloidosis: development of cardiac symptoms during 6 years of follow up in different ATTR-variants
Methods Clinical data and information with regard to survival was collected for all ATTR patients between 1994 and 2014 in the University Medical Centre Groningen, which is the Dutch national centre of expertise. In total 114 consecutive patients carrying 10 different TTR mutations were admitted. Patients were divided into different groups based on their mutation. Only mutations present in more...
متن کاملMulti-modality imaging in cardiac ATTR amyloidosis: agreement between echocardiography, MRI and DPD-scintigraphy
Methods Seventy seven consecutive patients with multimodality imaging evaluation (echocardiography, 1.5T MRI and 99mTc-DPD scintigraphy) to diagnose cardiac amyloidosis were identified from the database of the French National Reference Center for Amyloidosis. Patients with pacemaker or severe renal failure did not undergo cardiac MRI and were analyzed on the basis of the echocardiography and sc...
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ژورنال
عنوان ژورنال: Orphanet Journal of Rare Diseases
سال: 2015
ISSN: 1750-1172
DOI: 10.1186/1750-1172-10-s1-i10