Disphosphonates cardiac uptake in familial amyloid neuropathy: Comparison between DPD and HMDP
نویسندگان
چکیده
منابع مشابه
Disphosphonates cardiac uptake in familial amyloid neuropathy: Comparison between DPD and HMDP
Background Familial amyloid polyneuropathy (FAP) is a severe hereditary disease, due to production by the liver of a genetic variant transthyretin (TTR) resulting in tissue amyloid deposits. Cardiac involvement is of major prognostic value. Diphosphonate scintigraphy has been proposed as a diagnostic tool for TTR-related cardiac amyloidosis, but there is no consensus on the optimal radiopharmac...
متن کاملComparison of MIBG and Diphosphonate scintigraphy in cardiac involvement of aTTR-FAP
Background In familial aTTR amyloid polyneuropathy (FAP) cardiac involvement is of major prognostic value. Two approaches using radionuclide imaging proved relevant in the assessment of aTTR-related cardiac amyloidosis: detection of amyloid deposits with disphosphonates (DPD) and of sympathetic denervation with MIBG. The study aimed to compare the respective value of both approaches in patients...
متن کاملTongue atrophy and fasciculations in transthyretin familial amyloid neuropathy
Objective: Macroglossia is a well-known feature of amyloidosis; however, tongue atrophy and fasciculations are rarely seen and can lead to the misdiagnosis of amyotrophic lateral sclerosis (ALS). Methods: We identified 2 unrelated patients with atypical features of tongue atrophy and fasciculations in the setting of a severe neuropathy. Results: Both patients were confirmed to have transthyreti...
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چکیده ندارد.
15 صفحه اول(99m)Tc-DPD uptake reflects amyloid fibril composition in hereditary transthyretin amyloidosis.
Aims In transthyretin amyloid (ATTR) amyloidosis various principal phenotypes have been described: cardiac, neuropathic, or a mixed cardiac and neuropathic. In addition, two different types of amyloid fibrils have been identified (type A and type B). Type B fibrils have thus far only been found in predominantly early-onset V30M and in patients carrying the Y114C mutation, whereas type A is note...
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ژورنال
عنوان ژورنال: Orphanet Journal of Rare Diseases
سال: 2015
ISSN: 1750-1172
DOI: 10.1186/1750-1172-10-s1-p41